OBJECTIVE To study the clinical, pathologic and imaging features of multilocular cystic renal cell carcinoma (MCRCC) and to review the diagnosis and treatment of this subtype of renal cell carcinoma (RCC). METHODS The data from 8 cases (mean age, 49.4; 5 men and 3 women) who had been treated from 2004 to 2006, were reviewed retrospectively. Radiologic and pathologic documents were evaluated. For treatments, radical nephrectomy was conducted in 4 patients, partial nephrectomy in 2 and laparoscopic nephrectomy in 2.RESULTS Postoperative pathological findings confirmed the diagnosis of MCRCC. The stage of all 8 cases was pT1. For pathologic grade, 7 cases were G1 and 1 case was G2. Seven patients available for follow-up had survived tumor-free during the mean time of 8 months. CONCLUSION MCRCC is an uncommon subtype of RCC, it has a lower malignant potential and a better prognosis compared with other types of RCC. Nephron-sparing surgery may be an appropriate treatment options for MCRCC.