Meckel's diverticulum (MD) is the most common congenital anomaly of small intestine. It is the most common end result of the spectrum of omphalomesenteric duct anomalies, which also include umbilicoileal fistula, umbilical sinus or cyst, and a fibrous cord connecting the ileum to the umbilicus. Besides this, abnormal remnants of the urachus can present as patent urachus, vesicourachal diverticulum, urachal sinus or urachal cyst (UC). Urachal cysts represent an incomplete closure of the urachus, a cord-like structure connecting the primitive bladder to the umbilicus in early embryonic formation.Primary aim of this study was to present and share an orginal case of enterocutaneous fistula formation of MD via the UC in an adult patient, which is the first in literature.A 39-year-old male applied to the emergency surgery service with complaints of increasing abdominal pain, nausea, vomiting and fecal discharge from the umbilicus. Laparotomy was carried out with a suspicion of intrabdominal mass and enterocutaneous fistula formation. The surgical exploration revealed enterocutaneous fistula formation of MD via the UC. The patient was treated successfully with resection of UC and MD, and had an uneventful postoperative recovery. We conducted a literature review of studies published in the English language on Meckel's diverticulum and Urachal anomalies, accessed via PubMed, Medline, and the Google Scholar databases.Urachal enterocutaneous fistula through the umblicus in adult has not been encountered in the literature to the best of our knowledge. We present a original case and literature review.