腎細胞癌與血管肌肉脂肪瘤同時併存於結節性硬化症病人身上的情況非常少見。在此報告一個33歲男性結節性硬化症的病患，其腎臟同時包含有兩側血管肌肉脂肪瘤及左側腎細胞癌兩種不同的病灶。我們對其病史、診斷、治療及相關的文獻報告作一描述。

Renal cell carcinoma coexisting with angiomyolipoma in tuberous sclerosis is extremely rare. Hence a 33-year-old male patient is presented who was a victim of tuberous sclerosis with left renal cell carcinoma and bilateral angiomyolipoma. The history diagnosis, treatment and a brief review of literature are also described.