Pseudoprune-belly syndrome is a rare anomaly, an incomplete form of Prune-belly syndrome. The etiology is not clear, and though this syndrome may present associated with other malformations, the association with a liver cyst is extremely rare. We report the case of a male newborn who was referred to our hospital with an antenatal ultrasound that suggested a gastric duplication. Clinical examination revealed lax abdominal wall, unilateral undescended testis and a palpable mass on the right upper quadrant with fuzzy boundaries. On laparotomy we found a giant hepatic cyst displacing the bowel to the left. The baby underwent a partial ressection and marsupialization of the cyst as total resection was infeasible. The patient's recovery was uneventful. Although rarely, the Pseudoprune-belly syndrome can present with a liver cyst. Large liver cysts should be partially or totally ressected as soon as possible.

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