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Abstract Purpose  We report our experience of diagnosing and treating hepatic angiomyolipoma (HAML), a rare benign mesenchymal tumor. Methods  We analyzed retrospectively the clinicopathologic, radiological, and operative data of 25 patients who underwent surgery for HAML at our institute between November 2001 and May 2006. Results  Most patients (20/25) were asymptomatic and had normal liver function. Ultrasonography (US) showed a heterogeneous hyperechoic mass in 13 of 23 patients, precontrast computed tomography (CT) showed that all of 12 lesions scanned were hypodense, and magnetic resonance imaging (MRI) showed hypointensity on T1-weighted images and hyperintensity on T2-weighted images in most (5/6) lesions. Marked enhancement in the arterial phase was seen in 10 of 12 lesions on CT scans and in 6 of 6 lesions on MRI scans. All tumors were composed of varying proportions of smooth muscle, adipose tissue, and blood vessels, and showed positive immunohistochemical staining for HMB-45. All patients underwent partial hepatectomy and there was no evidence of recurrence after a median follow-up of 43 months. Conclusion  The radiological features of HAML vary according to its histological components. The definitive diagnosis of HAML is challenging and depends on the presence of HMB-45-positive myoid cells. Hepatic angiomyolipoma is treated effectively with surgery and the prognosis is good. [ABSTRACT FROM AUTHOR]