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Meckel diverticulum is the most common congenital anomaly of the small intestine, occurring in about 2%–4% of the population. Meckel diverticulum results from incomplete closure of the omphalomesenteric duct. The presentation of symptomatic Meckel diverticulum includes gastrointestinal hemorrhage, intestinal obstruction, volvulus, intussusception, diverticulitis, and neoplasms. The development of fistula is an extremely rare complication. Previous literature has even shown an enterocolonic fistula, a vesicodiverticular fistula, ileorectal fistula, and fistula-in-ano. To the best of our knowledge, we present the first case of the fistula complicated between Meckel diverticulum and the appendix in a review of the English literature.