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Two cases with angiomyolipoma were reported. The one was a 52-year-old housewife whose chief complaint was pain in the right abdomen. Her past history included adenoma cebaceum on the face, fits of epilepsy in her infancy, ichthysislike exanthems in lower thighs and kidney tumor on both sides on IVP. On the diagnosis of Bourneville-Pringle phacomatosis, she underwent an operation for the removal of the right kidney and partial resection of the uppermost part of the left kidney. Histopathologically, she was diagnosed as angiomyolipoma. The other case was a 36-year-old housewife whose chief complaint was pain in the right abdomen with cholelithiasis. In the course of the operation to remove the gallbladder, a right kidney tumor was detected accidentally, resulting in the concurrent removal of the right kidney. Histopathologically, a diagnosis of angiomyolipoma was made. The latter case was free from the Bourneville-Pringle phacomatosis. To the best of our collection, a total of 80 cases, including these cases, has been reported in Japan. A clinical statistic observation was made with priority given to these cases.