Adenomatosis is a rare lesion of unknown etiology, defined as multiple (usually 5 or more) adenomas in one kidney. A case of renal adenomatosis in a 68-year-old woman treated previously for urolithiasis, who underwent nephrectomy because of the nonfunctional left kidney is reported. Apart from multiple adenomas, numerous hyperplastic lesions involving single tubules were present in the resected kidney. Both adenomas and hyperplastic lesions exhibited the expression of alpha-methylacyl-coenzyme A racemase (AMACR). Renal adenomatosis is worth special attention, since renal papillary adenomas are suggested as precursor lesions of papillary renal cell carcinoma that show similar AMACR expression.