A case is described of a 58 year old Caucasian male with primary biliary cirrhosis (PBC) who first presented with acanthosis nigricans of both axillae, skin pigmentation, which was pronounced over the posterior surface of the neck, and generalized pruritus. Following orthotopic liver transplantation for progressive liver disease, the skin pigmentation, pruritus and acanthosis nigricans resolved. It is believed that this is the first reported case of acanthosis nigricans occurring in association with PBC, a phenomenon that resolved after liver transplantation.