We describe a 37-year-old man complaining of right back pain and gait disturbance. He had a big soft tumor on his right back, hemihypertrophy of the right lower extremity, and right thoracic scoliosis. We diagnosed Klippel-Trenaunay-Weber syndrome based on the pathological findings of the soft tumor. Computed tomography (CT) scan revealed severe spinal stenosis due to a hypertrophic vertebral body and facet joint at T7. Treatment by decompression of hypertrophic bone led to complete neurological recovery. To our knowledge, no case has been reported of Klippel-Trenaunay-Weber syndrome with myelopathy which originated from thoracic scoliosis with a hypertrophic facet joint and vertebral body. We suggest that the cause of myelopathy in Klippel-Trenaunay-Weber syndrome originated not only from arteriovenous fistula, medullary angioma, and extradural hemangioma but also vertebral hypertrophy with scoliosis.