OBJECTIVE: This study aims to analyze the prognosis of malignant peripheral nerve sheath tumors (MPNSTs) in children using the Surveillance, Epidemiology, and End Results (SEER) database to identify significant prognostic factors affecting survival. METHODS: A retrospective cohort analysis was conducted using the SEER database, identifying pediatric patients diagnosed with MPNSTs from 2000 to 2019. Demographic data, tumor characteristics, treatment modalities, and survival outcomes were extracted and analyzed. The main outcome measure was overall survival (OS), analyzed using Kaplan-Meier methods and Cox proportional hazards models to assess the impact of clinical and demographic factors on survival. Furthermore, we constructed a nomogram to predict OS in pediatric MPNSTs patients. RESULTS: The study included 208 pediatric patients with MPNSTs, with a near-equal distribution across gender and a majority being white. The most common sites for MPNSTs were the trunk and core areas (38.9%) and the limbs (34.1%). A majority of the patients (87.0%) underwent surgical treatment, and radiation therapy was administered to 44.7% of the patients. The overall 5-year survival rate for the entire cohort was 59.9%. When compared to no surgery, surgery had better survival outcomes. According to the results of Cox proportional hazard regression, only the SEER stage was an important independent predictor of OS in this model. CONCLUSIONS: Pediatric MPNSTs presents with a challenging prognosis, significantly influenced by the SEER stage at diagnosis. Surgery is crucial as first-line treatment for MPNSTs, especially if the tumor is localized at diagnosis.