Objective: To study the diagnosis and treatment of renal angiomyolipoma. Methods: Clinical data of 23 cases of renal angiomyolipoma were respectively analysed and discussed with review of the literature. Results: Of the 23 cases, 3 were misdiagnosed preoperatively. Operations were performed on 23 cases and the diagnosis was demonstrated pathologically. Preoperative selective renal-angioembolization and temporary renal arterial occlusion can reduce bleeding and avoid unnecessary nephrectomy significantly. Conclusions: A definite diagnosis of renal angiomyolipoma depends on ultrasound B and computed tomography. Most of the benign space-occupying lesions of kidney could be diagnosed with the combined use of B-ultrasonography, CT and Doppler ultrasonography. Frozen section pathological study during operation should be emphasized.