Renal malakoplakia (RM) with secondary hepatic involvement is extremely rare. There are only two cases reported in literature. We report the first case of RM with secondary hepatic extension in Korea. A 70-year-old woman had a history of long-term oral steroids intake. A radical nephrectomy and a segmental hepatectomy were performed under the clinical impression of a renal cell carcinoma with hepatic extension on the dynamic liver computed tomography. The gross specimen revealed a lobulated yellowish-tan, soft mass (9 X 8 X 5 cm) with hepatic involvement in the upper pole of the right kidney. The hematoxylin and eosin, Prucian blue and von-Kossa stainings, and electron microscopy demonstrated the classical histologic features of malakoplakia with Von Hansemann histiocytes containing Michaelis-Gutmann bodies. The patient is well 5 months after surgery. This case emphasizes that initial diagnoses can be difficult because of the rare occurrence of RM and its resemblance to renal cell carcinoma with hepatic extension.