Idiopathic adulthood ductopenia manifesting as jaundice in a young male

Idiopathic adulthood ductopenia (IAD) is an unusual disease of unknown etiology which results in chronic cholestatic liver disease. It manifests in young or middle aged adults and is characterized by absence of specific autoantibodies and a loss of interlobular bile ducts. It is a rare cause of cholestatic jaundice with less than 100 cases reported worldwide. We report a case of 18 year male patient who was diagnosed to be suffering from IAD based on clinical, pathological and radiological investigations.