Purpose: The treatment strategy for salivary gland acinic cell carcinoma in pediatric patients remains controversial. This retrospective study was undertaken to analyze the role of surgery in the treatment of acinic cell carcinoma of the major salivary gland in pediatric patients. Patients and Methods: In this retrospective cohort study, we reviewed the medical records of all pediatric patients with acinic cell carcinoma of the major salivary gland who were treated at Beijing Stomatological Hospital of Capital Medical University from 1998 to 2015. The predictor variable was treatment modality The outcome variables were disease-free survival (DFS), overall survival (OS), local control, and freedom from distant metastasis. Other variables of interest were as follows: age, gender, tumor site, T category, N category, recurrence history, pathologic grade, perineural invasion, extracapsular extension, positive margin, and resection condition. The data analysis methods used were descriptive, bivariate statistics and the Cox proportional hazards regression model. Results: Of the 19 patients, 7 received surgery alone and 12 received initial surgery combined with postoperative radiotherapy (RT). During the median follow-up period of 86 months, the overall estimates of DFS, OS, local control, and freedom from distant metastasis were 82.6, 93.3, 89.5, and 94.4%, respectively. Good outcomes were achieved in patients who received surgery alone (100% OS and 85.7% DFS). Initial surgery combined with postoperative RT was appropriate for patients with risk factors (91.7% OS and 83.3% DFS). Conclusions: Surgery alone is appropriate for salivary gland acinic cell carcinoma in pediatric patients without risk factors, even if extracapsular excision is adopted. Preserving the invaded facial nerve during surgery is a good choice because a curative effect can be obtained when adjuvant RT is administered. Older age, high-grade pathology, incomplete resection, recurrence history, and extracapsular extension were identified as risk factors of poor prognosis. (C) 2019 American Association of Oral and Maxillofacial Surgeons