Granulosa cell ovarian tumors are known to secrete estrogen. Herein we report a patient presenting with primary amenorrhea and virilization with markedly high androgen levels, all thought to be disproportional to be attributed to PCOS alone. Bilateral oophorectomy revealed a rare androgen secreting granulosa cell ovarian tumor and bilateral cysts (PCOS) both contributing to manifestations. Clinical Case: A 25 year old female presented with primary amenorrhea, male pattern baldness and hirsutism of the face and chest, clitoral hypertrophy, cystic acne on chest and shoulders and Type 2 Diabetes. Diagnosis of PCOS was made at age 13 years. Concurrent presence of congenital adrenal hyperplasia was also considered. She was receiving metformin, oral contraceptives and/or spironolactone with no improvement in manifestations at presentation to endocrine clinic. She reported several elevated serum testosterone and androstenedione levels. Diagnosis of PCOS was established by presence of enlarged cystic ovaries on ultrasound examination. Patient reported worsening manifestations including progression of Diabetes despite therapy with metformin. She shaved face, chest and breasts daily for cosmetic reasons. Laboratory testing demonstrated markedly elevated total testosterone (234 ng/dl), normal cortisol and ACTH levels. Dexamethasone suppression and HCG stimulation indicated ovaries as a probable source of excessive circulating androgen levels. CT scan of the abdomen and pelvis showed multiple cysts in both ovaries, largest being 1.9 cm in right ovary. Due to severity of manifestations, patient was counseled to undergo fluoroscopically guided blood sampling of bilateral adrenal and ovarian veins which confirmed both ovaries to be the source of the excess androgen production with a greater contribution by the right ovary. Patient underwent bilateral oophorectomy. Pathological examination confirmed the presence of bilateral polycystic ovaries as well as androgen secreting granulosa cell tumor in right ovary which apparently contributed to greater androgen levels in right ovarian venous blood sample. Peripheral venous androgen levels normalized promptly after bilateral oophorectomy. Gradual resolution of clinical manifestations followed. Conclusion: A unique presentation of granulosa cell ovarian tumor with concurrent polycystic ovarian syndrome contributing to the extremely excessive production of androgens in a young woman manifesting primary amenorrhea and masculinization at the onset of puberty with marked gradual resolution of manifestations following bilateral oophorectomy.