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International guidelines recommend referral for genetic testing immediately after diagnosis since the genetic result guides clinical management. We used next\u2010generation sequencing to identify a homozygous pathogenic variant, p.(Arg244*), in <jats:italic>COQ9<\/jats:italic> in 2 individuals referred for NDM testing. Both had insulin\u2010treated hyperglycemia, severe structural brain defects, dysmorphic features, and lactic acidosis. Recessive loss\u2010of\u2010function variants in <jats:italic>COQ9<\/jats:italic> cause Coenzyme Q10 deficiency\u20105, a multi\u2010system mitochondrial disease, with 7 cases reported. Neonatal hyperglycemia has not been reported in any of these cases but has been described for two other Coenzyme Q10 disorders caused by variants in <jats:italic>COQ2<\/jats:italic> and <jats:italic>COQ4<\/jats:italic>. Our report shows that individuals with <jats:italic>COQ9<\/jats:italic>\u2010related disease can present with neonatal hyperglycemia, expanding the clinical spectrum of this disorder. We recommend the inclusion of <jats:italic>COQ9<\/jats:italic>, as well as <jats:italic>COQ2<\/jats:italic> and <jats:italic>COQ4<\/jats:italic>, to gene panels used for NDM testing.<\/jats:p>","DOI":"10.1111\/jdi.70022","type":"journal-article","created":{"date-parts":[[2025,3,10]],"date-time":"2025-03-10T16:53:22Z","timestamp":1741625602000},"page":"959-963","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Two cases of neonatal hyperglycemia caused by a homozygous <scp><i>COQ9<\/i><\/scp> stop\u2010gain variant"],"prefix":"10.1111","volume":"16","author":[{"given":"Russell","family":"Donis","sequence":"first","affiliation":[{"name":"Department of Clinical and Biomedical Sciences, Faculty of Health and Life Sciences University of Exeter  Exeter UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maryam","family":"Al Badi","sequence":"additional","affiliation":[{"name":"National Diabetes and 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