Gender-variant individuals with somatic disorders of sex development (DSD) differ from gender-variant individuals without DSD in phenomenon, context of presentation, etiology, and treatment options. Over the last 15 years, medical and psychosocial management policies have been widely debated, and recent international consensus conferences have recommended significant modifications. Optimal care for children with DSD requires an experienced multidisciplinary team whose composition will vary with DSD category, age of patient, and local resources. Major gender-related aspects of the psychosocial management of children with DSD include gender assignment and reassignment, genital surgery, sex hormone treatment, and gender-relevant information management. These are the focus of the current paper. The psychosocial management of other aspects of DSD should take advantage of evidence-based approaches, where applicable, that have been developed for patients suffering from other congenital or chronic disorders.