Introduction: An omphalocele is a congenital malformation due to a failure of closure of the umbilical ring with exteriorization of the abdominal viscera covered by the translucent and avascular amniotic membrane. We report a case of giant omphalocele unrecognized at birth followed by a review of the literature. Observation: This was a 4-year-old girl admitted to the department for umbilical swelling that had been present since birth. Clinical and paraclinical examinations made it possible to objectivize a post-omphalocele hernia with hepatic content plus ectopic accession of the left ureter, the surgical procedure consisted of reintroducing the liver associated with a cure of the hernia + ureterovesical reimplantation. Conclusion: Omphalocele is a congenital malformation, antenatal diagnosis is possible during morphological ultrasound from the 12th week.
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