The authors present a case of Frasier syndrome in a 17-year-old girl with nephrotic syndrome and male pseudohermaphroditism. Due to the existing risk of developing tumors in dysgenetic gonads, the patient was admitted to the clinic for prophylactic gonadectomy The operation was then postponed as a result of rapid progression to end-stage renal failure, and the patient was placed on hemodialysis. During subsequent laparoscopy both ovaries and dysgenetic gonads were resected. Histopathological examination revealed the presence of both seminal ducts and epididymis. Early prophylactic resection of dysgenetic gonads, such as was undertaken in this patient, is indicated in children with Frasier syndrome to prevent the development of germ cell tumors.