血管平滑肌脂肪瘤 (angiomyolipo-ma,AML)常发生于肾脏。发生于肝脏者十分罕见。自 1976年 Ishak等 [1 ]首先报道以来 ,国内外累计报道约 70例 ,主要为个案报道 ,国内报道极少。术前诊断十分困难。本文就其临床表现、影像学特点、病理特征及治疗方法等方面进行综述 ,以提高对该 Angiomyolipo-ma (AML) often occurs in the kidneys. Occur in the liver are very rare. Since the first report by Ishak et al [1] in 1976, about 70 cases have been reported at home and abroad, mainly for case reports and very few reports in the country. Preoperative diagnosis is very difficult. This article reviews its clinical manifestations, imaging features, pathological features and treatment methods to improve the