OBJECTIVE Multilocular cyst of kidney is a rare, unique and characteristic renal lesion with benign biologic behavior. We report a case of the disease. METHODS It was confirmed by operation and pathology. RESULTS It is difficult to distinguish radiologically multilocular cyst of kidney from cystic renal carcinoma and wilms tumor. CONCLUSIONS Surgical intervention is the only effective treatment and method to differentiate multilocular cyst of kidney from a malignant lesion of the kidney.