Abstract
The authors report a preterm infant with Rh-isoimmunization, who had persistent hepato-splenomegaly with conjugated hyperbilirubinemia, transaminitis, and hyperammonemia. Ultrasound abdomen revealed an intrahepatic portosystemic venous shunt (PSVS). The child was managed conservatively. On follow up at 2.4 y of age, the child is having normal growth and development, but with persisting shunt. Severe Rh-isoimmunisation in a neonate can sometimes share some of the features of congenital PSVS and delay the diagnosis of the latter. The index case had shunt ratio >80 % during the neonatal period but did not require any intervention.
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