A 49-year-old woman with primary Sjögren's syndrome a few years previously was admitted to our hospital complaining of tongue and skin eruptions, swelling of the face and neck and for examination of liver injury and hypereosinophilia. A blood test revealed leukocytosis with eosinophilia, mild liver injury, polyclonal hypergammaglobulinemia, and positive results for anti-nuclear antibody, anti-SS-A antibody and anti-SS-B antibody. Chest CT scan showed multiple nodular opacities with cavities in peripheral regions of both lungs. Biopsy specimens from the right lower lobe obtained by video-assisted thoracoscopy revealed marked infiltration of plasma cells and lymphocytes in alveolar lumina, lymph follicles with germinal centers in other areas of the pulmonary parenchyma, and lymphocytes infiltrate in alveolar wall adjacent bronchi and bronchioles. The histological diagnosis was pulmonary involvement of multicentric Castleman's disease. This was a rare case of Sjögren's syndrome accompanied by pathological findings of multicentric Castleman' s disease showed multiple nodular opacities in chest CT scans.