Disorders of sex development (DSD) refer to an extensive range of conditions wherein the chromosomal, gonadal or phenotypic sex is incongruous. Presented is a case of ambiguous genitalia in a 3 year old child reared as female since birth and subsequently assigned a male gender after being diagnosed with 46,XY DSD. Management ideally involves a multidisciplinary approach to address the myriad of issues in the care of a child with DSD and to address significant concerns including: gender assignment and gender identity; timing of medical and surgical interventions and equally important, psychosocial and psychosexual development. Current recommendations revolve around patient-centered care which advocates respect for the autonomy and personhood of the patient by postponing the making of crucial decisions, whenever possible, until the child is capable of participating in the decision-making process.