Gonadal dysgenesis (46,XY) and gonocytoma

A case of 46,XY gonadal dysgenesis with a type III Teter's gonocytoma occurred in the left ovary of a 22-year-old woman. Hormonal, genetic and laparoscopic studies were performed before the diagnosis of gonadal dysgenesis was made. Gonadal neoplasm was found after bilateral gonadectomy.