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We present a case of a transfusion\u2010dependent male newborn who presented at birth with severe hemolytic anemia, and required an intrauterine transfusion. Genetic testing rapidly identified a Kruppel\u2010like factor 1 (KLF1) pathogenic variant (c.973G&gt;A, p.E325K), known to be causative for CDAIV. This case highlights the advantages of next\u2010generation sequencing testing for congenital hemolytic anemia: diagnostic speed, guidance on natural history, and optimized clinical management and anticipatory guidance for parents and clinicians. Additionally, we reviewed the literature for all CDAIV cases.<\/jats:p>","DOI":"10.1002\/pbc.30245","type":"journal-article","created":{"date-parts":[[2023,2,17]],"date-time":"2023-02-17T01:23:26Z","timestamp":1676597006000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Congenital dyserythropoietic anemia type IV in the genetic era: A rare neonatal case report of rapid identification with a review of the literature"],"prefix":"10.1002","volume":"70","author":[{"given":"Marc\u2010Olivier","family":"Deguise","sequence":"first","affiliation":[{"name":"Faculty of Medicine University of Ottawa  Ottawa Ontario Canada"},{"name":"Department of Pediatrics Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Blain","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of Ottawa  Ottawa Ontario Canada"},{"name":"Division of Pediatric Hematology\/Oncology Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ewurabena","family":"Simpson","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of Ottawa  Ottawa Ontario Canada"},{"name":"Department of Pediatrics Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"},{"name":"Division of Pediatric Hematology\/Oncology Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mira","family":"Liebman","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of Ottawa  Ottawa Ontario Canada"},{"name":"Department of Pediatrics Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"},{"name":"Division of Pediatric Hematology\/Oncology Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Ferretti","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of Ottawa  Ottawa Ontario Canada"},{"name":"Department of Pediatrics Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"},{"name":"Division of Neonatology Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,2,16]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019000948"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-020-03982-y"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.exphem.2019.03.001"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.13097"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.25058"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000001056"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000001042"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ccr3.825"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2013.02.006"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2010.10.010"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1991.tb04541.x"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000001727"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12864-019-5805-z"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.00444-19"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mrrev.2020.108336"},{"issue":"1","key":"e_1_2_9_17_1","first-page":"s302","article-title":"A case of severe foetal anaemia due to anti\u2010M isoimmunisation salvaged by intrauterine transfusions","volume":"12","author":"Bajpayee A","year":"2014","journal-title":"Blood Transfus"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.3390\/jpm10040268"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-08-590968"},{"key":"e_1_2_9_20_1","volume-title":"Nathan and Oski's Hematology and Oncology of Infancy and Childhood","author":"Orkin SH","year":"2015"}],"container-title":["Pediatric Blood &amp; 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