Abstract Background Disorders of sex development (DSD), which has the possibility of the risk of life-threatening endocrinologic emergencies of the newborn, require a careful multidisciplinary approach. Objectives The aim of our study is to consolidate the proposed classification, evaluation and management of DSD. Materials and methods The literatures related with DSD were reviewed to find the best approach for this disease. Results The detailed history, systemic physical examination of the patient, particular laboratory and imagine evaluations are needed for the urgent treatment of life-threatening abnormalities and the gender assignment. Conclusion The gender should be assigned depending on the definitive diagnosis, fertility potential, genital appearance, surgical options, and the parents’ opinion.