Idiopathic adulthood ductopenia is a chronic cholestatic entity of unknown origin that was discovered in the past decade. Although it is classically a progressive and severe disease, a benign and responsive-to-therapy form has been reported recently. We present two middle-aged females that represent the extremes of the clinicopathologic spectrum: One with a severe form of the disease for whom liver transplantation was considered and the other with a benign form.