A prospective descriptive study of Primary Biliary Cirrhosis at Hospital Kuala Lumpur was undertaken from January 1992 to December 1999. A total of 17 patients were seen with a female to male ratio of 3.25:1. The mean age at presentation was 45.9 years (range: 14 years to 67 years) with a mean follow-up of 33.4 months (range: 3 months to 95 months). Fatigue was the most common clinical symptom at presentation. Alanine transaminase and alkaline phosphatase levels were elevated in 93% of patients at presentation. The antimitochondrial antibody was positive in 87% of patients. Ursodeoxycholic acid therapy resulted in significant symptomatic relief and biochemical improvement in all those who were treated.