An eight-month-old female, delivered to consanguineous parents, presented with acute liver cell failure. Her investigations showed progressive cholestatic jaundice, high liver enzymes and high gamma-glutamyl transferase. Hepatitis and inborn errors of metabolism were excluded. The liver biopsy showed a prominent parenchymal bile stasis without features of bile obstruction or paucity of bile ducts. These findings wee suggestive of Byler disease or progressive familial intra hepatic cholestasis type III (PFIC III) which begins in infancy and usually progresses to cirrhosis and hepatic failure in the first few years of life.