Renal pelvis choriocarcinoma is rare. Most reported cases occurred in females secondary to metastasis from the primary lesion. Here we report a case of renal pelvis choriocarcinoma with renal stone in a 67-yr-old male. Upon physical examination, there was no definite testicular abnormality, and image studies revealed a right renal pelvis tumor with multiple metastasis of the tumor. The tumor was temporarily responsive to chemotherapy after percutaneous biopsy proved choriocarcinoma. The origin of primary renal pelvis choriocarcinoma remains unknown although transitional cell carcinoma with choriocarcinomatous differentiation has been proposed.