淋巴血管肌肉增生症是罕見的、病因未知的疾病，其通常發生在生育年齡的女性和患有結節硬化症的人。在淋巴血管肌肉增生症，腹腔內表現最常見的是腎臟血管肌肉脂肪瘤，而且大部分是沒有症狀和小的。我們報告一個六十一歲的女性淋巴血管肌肉增生症患者主訴左腹逐漸增大，腎臟腫瘤切除後，病理報告為巨大的血管肌肉脂肪瘤。

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown origin that usually occurs in women of reproductive age and people with tuberous sclerosis. Although renal angiomyolipomas are the most common intraabdominal manifestation of the disorder, the majority are asymptomatic and small. We report a LAM in a 61-year-old female with progressive enlargement of left abdomen. The renal tumor was resected and a huge angiomyolypoma was diagnosed by pathological examination.