PurposeTo investigate the clinical features and survival outcomes of primary gastrointestinal non-Hodgkin lymphomas (PGINHL) in pediatric and adolescent population, we conducted a population-based cohort study.MethodsAll pediatric and adolescent patients with PGINHL diagnosed between 2000 and 2019 were identified using the Surveillance, Epidemiology, and End Results (SEER) database. Kaplane-Meier estimations were used to generate survival curves based on various criteria. To compare survival curves, the log-rank test was applied. A multivariate Cox proportional hazards model was developed to investigate the effect of each component on overall survival.ResultsA total of 334 pediatric and adolescent with PGINHL patients were identified. The median age at diagnosis was 12 years (range 1.0–19 years). Tumors were most commonly found in the small bowel (47.3%), followed by the large bowel (42.8%) and the stomach (9.9%). Overall, the most common histological subtype was Burkitt lymphoma (56.9%), followed by diffuse large B-cell lymphoma (DLBCL) (27.8%). Overall survival rates for all patients were 92.2% at 5- year and 91.6% at 10- year, respectively. The Cox proportional hazard regression revealed that only chemotherapy was an important independent predictor in this model. Patients with chemotherapy have a higher survival rate than those without.ConclusionsOur study revealed that only chemotherapy was found to be the most important predictor of the OS in pediatric and adolescent PGINHL, providing critical information for therapeutic care.