The case of a 29-year-old man with Niemann-Pick disease and hepatic failure is presented. Massive hepatomegaly with hepatic calcification were noted in association with a course of persistent hepatitis B serum antigenemia with rapid hepatic decompensation, ascites, encephalopathy and renal failure. The possible relationship of the clinical course to the underlying disease process is discussed, and a review of Niemann-Pick disease is presented.