About 5% of dysgerminomas arise in gonadoblastomas occurring in phenotypic female with abnormal gonads. In Pure Gonadal Dysgenesis, the patients have bilateral streak gonads and 46, XY chromosomes and the risk of malignancy is 28% by age 20 years. Those with Mixed Gonadal Dysgenesis have unilateral streak gonad, contralateral testis, and 45,X/46,XY chromosomes with an estimated 19% risk of malignancy by 20 years of age. Therefore, patients with gonadal dysgenesis must undergo prophylactic removal of gonads at an early age. The image shows a large dysgerminomas with soft, fleshy, pink-tan cut surface and areas of hemorrhage and necrosis.