We herein report the first Japanese case of vertebral sarcoidosis diagnosed using multiple imaging modalities and a biopsy. CT, MRI and (18)F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) detected multiple vertebral lesions, and a vertebral biopsy guided by the PET findings confirmed the diagnosis of vertebral sarcoidosis. Although the disease was refractory to corticosteroids, treatment with methotrexate (MTX) achieved a good response. Our case suggests that MRI and 18F-FDG PET are useful for determining the site for a biopsy and that MTX is effective for treating vertebral sarcoidosis.