Esotracheal fistula is a rare malformation represented by a thin ascending channel between the esophagus and the posterior surface of the trachea. Diagnosis is sometimes difficult due to the atypical character of the symptomatology. Diagnosis is made by a gastro-duodenal oesophageal transit (TOGD) and the treatment is surgical.We report a case of isolated congenital esotracheal fistula collected in the pediatric visceral and urogenital surgery department at the Mohammed VI University Hospital Center in Oujda, Morocco, previously not discovered, and its surgical treatment as well as an updated literature review of this entity.