Cases of patients with cystic partially differentiated nephroblastoma (CPDN) have been reported to date, which presented as polycystic renal tumor in all of them. It is a special pathological type of nephroblastoma. Here, we report the first case of a unicystic CPDN in a child. The patient was diagnosed with a simple renal cyst and underwent laparoscopic decortication. The naive nephron was found in the pathological section, and the diagnosis of CPDN was confirmed. The patient then underwent a radical nephrectomy and six cycles of postoperative chemotherapy. There was no recurrence or metastasis after 2 years of follow-up. Pediatric CPDN presenting as a unicystic renal tumor poses a new challenge to the diagnosis, differential diagnosis, and treatment of unicystic renal tumor.