Disorders of sex development (DSD) are a conglomerate of rare diseases with an estimated incidence of 1: 4500. The causes of DSD are mainly disorders with gonadal dysgenesis, decreased androgen synthesis or function in XY males or disorders with elevated androgen production in XX females. Decision on sex of rearing is difficult in some cases as the prenatal androgen imbalances result in ambiguous genitalia at birth and furthermore they are likely to influence psychosexual development. Genital constructive surgery is needed in most cases. Lifelong cortisone replacement is needed in DSD due to defects of cortisone synthesis. Sex hormone substitution is indicated in many cases of DSD in puberty and adult life. Decision of sex of rearing, genital surgery and hormone therapies have a life-long impact on the affected individuals, which become evident mainly after puberty. In many cases psychological counselling is advised. Interpretation of previous outcome studies of DSD is hampered by small patient numbers and conglomerates of diagnoses and therapies. The study DSD-Life investigates and compares the long-term outcome of different off-label treatments in adequate numbers of adolescents and adults with different known genetic entities of DSD to develop recommendations for treatment of DSD. To reach this aim, the influences and interrelations of sex assignment, genital surgery, hormone therapy, metabolism, fertility, psychological intervention but also cultural influences and patients’ and parents’ views on psychosocial adaption, health related quality of life and psychological well-being and will be investigated. The long-term impact of the study will be improvement of care and subsequently higher quality of life with better integration and participation of individuals with DSD in the society.