Abstract
A baby with an antenatal diagnosis of exomphalos was born at term. The abnormality had a highly unusual appearance, a right-sided paraumbilical defect, similar to gastroschisis, but with a sac typical of exomphalos containing both abdominal contents and over 1 L of serosanguinous fluid. The sac was drained and suspended from the top of the incubator in silo fashion. Definitive closure was achieved at day 3 of life, and the patient had a non-eventful recovery. Despite exomphalos and gastroschisis normally being discrete entities, ambiguity in the presentation this case necessitated a composite management approach.