Arteriohepatic dysplasia or congenital paucity of interlobular bile ducts - Alagille Syndrome, is a well defined syndrome characterized by five major features, including chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects, peripheral pulmonary artery hypoplasia or stenosis and facial dysmorphy. The disease is very rare. Only three cases have been reported in Greece and none with renal involvement. Hepatobiliary scan was a fundamental tool in the patients diagnosis and therefore we present the following case.