Aim of the Study: To describe the clinical, surgical, and pathological features of children with acute appendicitis and amebiasis.Materials and methods: The medical records of children diagnosed with appendicitis and amebiasis treated at Fundación Hospital la Misericordia were retrospectively reviewed. Patients were classified into two groups according to the location of the amoebas: the amoebic appendicitis (AA) group (when the amoebic infection involved the appendiceal wall), and the appendicitis with incidental amoebiasis (IA) group (when amoebas were only found in the appendiceal lumen). We compared demographics and outcomes.Results: We identified 23 children with appendicitis and amoebiasis. The mean age was 9 (3–15) years, and 52% were male. The main duration of the pain at the time of presentation was 1.8 (1–4) days. Fever and diarrhea were observed in 64% and 43% of the patients, respectively. Four patients had perforated appendicitis, all of them within the AA group. Anti-parasitic therapy was used only in 2 subjects (all other patients were diagnosed after discharge and were asymptomatic on follow up). AA was diagnosed in 11 patients and IA in 12. AA and IA patients shared similar characteristics regarding age, clinical findings, radiologic features, and preoperative laboratory results. Appendiceal necrosis and perforation were only found within the AA group (4 patients).Conclusions: AA is a rare condition in children. It seems to have a greater risk of perforation than cases of appendicitis with IA, which is in agreement with the more severe histological findings in our series. Anti-parasitic treatment is not necessary in cases of AA or IA.