As part of the development of the eleventh revision of the International Classification of Diseases (ICD-11), WHO appointed a Working Group on Sexual Disorders and Sexual Health to recommend changes necessary in the classification of mental and behavioural disorders in ICD-10 that are related to sexuality and gender identity. This Personal View focuses on the Working Group’s proposals to include the diagnosis gender incongruence of childhood in ICD-11 and to move gender incongruence of childhood out of the mental and behavioural disorders chapter of ICD-11. This article, by Jack Drescher, Peggy T Cohen-Kettenis, Geoffrey M Ree, outlines the history of ICD and DSM child gender diagnoses, expert consensus, knowledge gaps, and controversies related to the diagnosis and treatment of extremely gender-variant children. It argues that retaining the gender incongruence of childhood category is justified as a basis to structure clinical care and to ensure access to appropriate services for this vulnerable population, which provides opportunities for education and informed consent, the development of standards and pathways of care to help guide clinicians and family members, and a basis for future research efforts.