Multilocular cyst of the kidney is an uncommon benign renal neoplasm. Because of its benign nature, this lesion is best managed by nephron-sparing surgery. This report describes a child who underwent a partial nephrectomy for a multilocular cyst of the kidney. After reviewing other cases, we discuss the clinical characteristics of multilocular cyst of the kidney and emphasize the cystic partially differentiated nephroblastoma as a differential diagnosis.