Disorders of somatosexual development that lead to ambiguous genitalia occur in one from 3,000-5,000 newborns. Parents and health care professionals are confronted with a number of crucial questions: to what sex should the child be assigned, what is the appropriate treatment in terms of hormonal and surgical interventions, when and how should these take place, and what impact do they have on the development of gender identity (GI), psychosexual well-being and fertility? This paper reviews the etiology, treatment and outcome in terms of GI and sexual health for the following syndromes: congenital adrenal hyperplasia (CAH), complete and partial androgen insensitivity (cAIS, pAIS), and pure and mixed gonadal dysgenesis (pGD, mGD). Emphasis is focussed on the current discussion involving the timing and extent of genital surgery. Finally, a procedure is introduced that covers the sexual-medical needs of patients, parents and health care professionals.