Collecting duct carcinoma (CDC) accounts for less than 1% of all renal carcinomas. It is a rare and aggressive neoplasm presenting generally at an advanced stage, and thus has a poor prognosis. The present case describes a 31-year-old female suffering from CDC coexisting with papillary renal cell carcinoma (PRCC) as well as liver, nodal and vertebral metastases. The patient was treated with open nephron sparing surgery. To date, there are only several clinical studies of CDC and few reports of synchronous CDC and PRCC in one kidney.