A 57-year-old woman who had shown an elevated level of biliary tract enzymes for 1 year claimed a persistent dry cough for 1 month. A chest radiograph showed bilateral patchy infiltrations in the lower fields, and she was admitted to our hospital. Transbronchial lung biopsy showed noncaseating epithelioid cell granuloma. We expected to diagnose her with pulmonary sarcoidosis according to the elevating serum angiotensin-converting enzyme level and the negative result of a tuberculin skin test, but the opacities in the lower fields predominancy without hilar lymphadenopathy, normal CD4/8 ratio of bronchoalveolar lavage fluid, and pulmonary eosinophilic infiltration and with the absence of network formation made this case atypical as pulmonary sarcoidosis. Those findings, such as high levels of serum antimitochondrial M2 antibody, lymphocyte infiltrations in the portal regions, disappearance of bile ducts, proliferations of cholangioles, partial epithelioid cell granuloma and eosinophilic infiltrations around the affecting areas, resulted in our diagnosing her with class II to class III primary biliary cirrhosis (PBC) (Scheuer classification). This case was suspected to be pulmonary involvements associated with PBC rather than comorbidities with pulmonary sarcoidosis because it presented with epithelioid cell granuloma and eosinophilic infiltrations in both the lung and the liver.