Idiopathic adult ductopenia is a rare disease characterized by chronic cholestasis related to an unexplained loss of the interlobular bile ducts. We report on a 43-year-old woman, whose clinical course of cholestatic disease was indolent and non-progressive, and laboratory abnormalities occurred intermittently. Histological examination of liver specimen showed absence of interlobular bile ducts, intensive proliferation of peripheral bile ductules and only minimal inflammatory portal response. Given the presence of microscopic non-specific chronic proctitis, hypothyroidism and antinuclear antibodies in serum we hypothesize that idiopathic adult ductopenia could be a variant of primary sclerosing cholangitis affecting exclusively small bile ducts.