Background: Neonatal ascites is a rare entity, which is usually biliary, urinary, or chylous. Congenital hepatic fibrosis as part of the manifestations of autosomal recessive polycystic kidney disease (ARPKD) is usually a histological adjunct to the diagnosis of this mainly clinically renal entity in the neonatal period. Case-diagnosis: We describe a rare case of severe ascites in a newborn child complicating ARPKD, which was later confirmed by clinical, histological, and genetic studies.
Conclusion: ARPKD should be considered in differential diagnosis of neonatal ascites.
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