Kaposiform hemangioendothelioma (KHE) is a rare locally aggressive vascular tumor that usually presents as a superficial or deep soft tissue mass with associated cutaneous lesions. We report a unique spinal KHE with painless thoracic scoliosis in a 14-year-old girl. She underwent simultaneous tumor biopsy, spinal deformity correction and fusion. At 3 years follow-up, the patient’s MRI showed no significant deterioration of process without any therapy. KHE presenting as scoliosis is rare and to our knowledge this is the first recognized case in the reported world literature.