Neuroblastoma arises from the embryonic tissue of the adrenergic rest. It is commonly found in children and mostly in nonrenal tissue. We present a case of intrarenal neuroblastoma which was initially thought to be a Wilms' tumor. The patient was a 18 months-old girl treated with radical nephrectomy and adjuvant chemotherapy after operation. The neoplasm within the kidney in children cannot always indicate Wilms' tumor. Neuroblastoma of the adrenal gland or retroperitoneal tissue may often compress or invade the kidney directly or arise from the kidney. Clinical aspects that differentiate between neuroblastoma and Wilms' tumor are discussed with a review of the literatures.