PURPOSE: Incidence of BS primitive neuroectodermal tumors (BS-PNET) in children is not reported to date. Our main objectives were to estimate the incidence and report the outcome of BS-PNET in children. METHODS: Data was collected using the Surveillance Epidemiology and End Results cancer registry. RESULTS: From 1973 till 2013, we identified 83 pediatric patients (aged 0–21 years). Patients were divided into 2 age groups (0–3 years and 4–21 years). Median overall survival was 53 months. Patients in the older age group had a significant survival advantage (P <0.001), as did those who received 3 modalities of therapy (surgery, chemotherapy, and radiation therapy) (P <0.001) and patients with gross or subtotal tumor resection (P <0.001). CONCLUSIONS: This study presents the first estimate of incidence and the largest cohort of pediatric BS-PNETs to date. A high index of suspicion of BS-PNET in similar cases is crucial for diagnosis, treatment, and outcome.