Tuberous sclerosis(TSC) is an inherited autosomal dominant disease. Many systems in the body are affected. However, the occurrence of malignancy in these patients is considered very rare, the most common being renal cell carcinoma. The occurrence of high grade papillary serous carcinoma(HGSC) involving the adnexa is extremely uncommon with hardly any literature available regarding the occurrence of this malignancy in TSC patients. A 62 year old female presented radiologically with mass near the uterus involving the left tube and ovary measuring 13x10x8cm and multiple cysts in the lung diagnosed radiologically as lymphangioleiomyomatosis. Radiologically the left kidney showed a lesion measuring 14x9x7cm and the right kidney showed angiomyolipomas. USG guided biopsy was performed in the left kidney and adnexa. Following this Left radical nephrectomy along with total hysterectomy with bilateral salpingo oophorectomy(TAH with BSO) was performed. Left kidney showed a lesion measuring 16x9x10cm which was histologically diagnosed as angiomyolipoma. TAH with BSO specimen showed a 13x10x8cm lesion occupying the left adnexa. The HPE was suggestive of a HGSC. The diagnosis and treatment of TSC is complex and requires a multidisciplinary approach involving various specialists to decrease the mortality and morbidity and thus increase their survival rate