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In this study, we report a 16\u2010year\u2010old boy with intramucosal gastric signet ring cells in the setting of a germline <jats:italic>CDH1<\/jats:italic> PV and a family history of early\u2010onset gastric cancer. The approach to managing both the proband and their 9\u2010year\u2010old sister, who also had the <jats:italic>CDH1<\/jats:italic> PV, presented a challenge to both clinicians and the family. Herein, we present the complexities of managing gastric cancer risk when a <jats:italic>CDH1<\/jats:italic> PV is identified in childhood in the setting of a family history of early\u2010onset gastric cancer.<\/jats:p>","DOI":"10.1002\/ajmg.a.63897","type":"journal-article","created":{"date-parts":[[2024,10,11]],"date-time":"2024-10-11T10:58:11Z","timestamp":1728644291000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Managing <scp><i>CDH1<\/i><\/scp> Cancer Risks in a Child: Complex Decision Making in a Family With Hereditary Diffuse Gastric Cancer"],"prefix":"10.1002","volume":"197","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9336-0552","authenticated-orcid":false,"given":"Nihat Bugra","family":"Agaoglu","sequence":"first","affiliation":[{"name":"Division of Cancer Genetics and Prevention Dana\u2010Farber Cancer Institute  Boston Massachusetts USA"},{"name":"Department of Medical Genetics, Division of 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