Introduction: Hepatic tumors in children are relatively rare, accounting of %1-4 of all pediatric solid tumors and infantile hemangioendothelioma (IHE) is the most common type of vascular tumors in infancy. There is female predominance and most of the patients are diagnosed during the first months of life. The natural course of disease varies, from spontan regression to heart failure and even death. Progressive familial intrahepatic cholestasis (PFIC) refers to a heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis. The signs of PFIC appears in the first years of life and leads to death from liver failure. Thus the liver transplantation is the only treatment option for these patients.

Case Report: We report a 7 months male patient, with glucose 6 phosphate dehydrogenase defficiency (G6PDD) and PFIC-2 leading to chronic liver failure and underwent orthotopic liver transplantation (OLT). On gross examination of his native liver; 0.5 cm nodule was recognised on posterior of right lobe, 2 cm away from capsule and was diagnosed as IHE histopathologically. Besides this nodule all the parenchyme showed submassive hepatocyte loss and diffuse hepatocanalicular cholestasis . The size of IHE nodule in our case was too small to make an abdominal mass and it was not recognised by abdominal sonography or computed tomography (CT) before transplantation. There were symptoms and signs like jaundice, anemia hepatomegaly, intraabdominal massive fluid, high AFP and liver enzymes in serum but all were explained by the PFIC-2 and G6PDD. For 3.5 years he was followed and didn’t have any rejection episodes.

Conclusions: We report this case because of rarity of PFIC-2 with IHE synchronously and review the literature focusing on IHE.