A 63-year-old man consulted our hospital for further examination of a solitary pulmonary nodule. Lung nodules increased in size and number after a few months. Fluorodeoxyglucose positron emission tomography (FDG-PET) showed a strong accumulation of the left internal juglar lymph node. A pathlogical diagnosis of the lymph node was histiocytic necrotizing lymphadenitis. Treatment with prednisolone (20 mg/day) was started, but multiple lung nodules gradually progressed. A video-assisted thoracoscopic (VATS) lung biopsy of the left lower lung nodule was performed. The histological findings showed angiocentric lesions with infiltration of polymorphous cells, and many atypical lymphocytes were positive for Epsterin-Barr virus-encoded small RNA by an in situ hybridization. Lastly, we diagnosed lymphomatoid granulomatosis grade III. Multiple lung nodules responded to chemotherapy with rituximab.