A 68-year-old woman was admitted to a hospital with pulmonary hypertension (PH) in August 2006. Perfusion scintigraphy of the lung was normal and showed no interstitial change. Liver dysfunction was noted and antinuclear antibodies (x1,280) were positive. In November 2006, muscle pain and weakness gradually developed in the brachial muscle and a quadriceps. She was referred and admitted to our hospital for elevated CPK and liver dysfunction in March 2007. She was diagnosed with polymyositis (PM) on the basis of the histological findings of muscle biopsy and treated with prednisolone. In addition, because anti-centromere antibodies and anti-mitochondrial M2 antibodies were positive with high titers, she was also diagnosed with primary biliary cirrhosis (PBC). Although PBC is often associated with other autoimmune diseases, there have been no reports of PBC complicated by PM and PH.