Megacolon, resulting in severe constipation, is one of the most common manifestations of Chagas' disease. We herein report a case of megacolon with fecaloma in a 39-year-old Brazilian man who presented with severe constipation. He underwent a total proctocolectomy and ileal J-pouch anal anastomosis. Histologically, there was a marked thickening of the proper muscle layer, and a moderate decrease in the myoenteric nerve plexuses and neurons in the megacolon area. A feasible outcome has been achieved after a 1-year follow-up. A total proctocolectomy with ileal J-pouch anal anastomosis may therefore be one of the useful strategies for the surgical treatment of patients with chagasic megacolon.