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 were collected using the Surveillance Epidemiology and End Results cancer registry. Results From 1973 to 2013, we identified 83 pediatric patients (aged 0–21 years). Patients were divided into two 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 three 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.