Introduction: Schwannomas are encapsulated nerve sheath tumors derived from Schwann cells, primarily found in the head, neck, and extremities. Retroperitoneal schwannomas are rare and typically asymptomatic.
Case Presentation: A 36-year-old female presented with a long-standing, asymptomatic right lower abdominal mass.
Key Clinical Message: In babies presenting with an omphalocele, other components of the prune belly syndrome should be scrutinized for early diagnosis and timely intervention.
Abstract: A male baby on his 13th day of life presented with an omphalocele. On evaluation, he had congenital absence of left kidney and bilateral cryptorchidism.