Background: Ameloblastoma is a benign but locally aggressive odontogenic tumor of the jaw, which is rare in pediatric populations. The clinical behavior, diagnosis, and management in children often differ from adults due to developmental considerations. Objective: This systematic review aims to summarize the clinical features, diagnostic methods, treatment strategies, and outcomes of ameloblastoma in pediatric patients. Methods: A comprehensive literature search was conducted in databases including PubMed, Scopus, and Web of Science. Studies on ameloblastoma in individuals ≤18 years published up to November 2024 from January 2020 were included. Data were extracted and synthesized qualitatively.Results: Pediatric ameloblastomas most commonly affect the mandible, presenting as painless swelling or facial asymmetry. Radiologically, they exhibit multilocular radiolucencies. Histologically, follicular and plexiform variants are predominant. Treatment includes conservative approaches like enucleation and curettage or radical resection, balancing recurrence risk and growth potential. Recurrence rates vary, with conservative treatments showing higher recurrence.Conclusion: Management of pediatric ameloblastoma requires individualized approaches considering the patient’s age, tumor size, and growth potential. Future research should focus on long-term outcomes and recurrence prevention strategies.