Ambiguous genitalia, currently defined as disorders of sex development (DSD), are not uncommon in the Muslim community. DSD constitute a complex, major social and medical emergency, as several forms of congenital adrenal hyperplasia can lead to significant salt loss, which may lead to shock if unrecognised and not appropriately treated. To ensure that the affected individual has a high quality of life (a successful outcome), medical practitioners must quickly and correctly assign the individual’s gender and effectively assuage the family’s concerns and anxieties. It is important to review and understand the embryology and physiology of sexual differentiation, and to understand the various aetiological causes of sexual ambiguity. In this review, the diagnostic approach and management of ambiguous genitalia is thoroughly discussed from an Islamic point of view.