Lymphangiomas are a group of benign malformations of the lymphatic system, and hemolymphangioma (or hemangiolymphangioma) is a rare congenital malformation of the lymphatic system with vascular involvement. These benign malformations are most commonly diagnosed at an early age, and may be present as a part of an associated syndrome. In this case report, we describe the first case of adolescent scoliosis associated with a large, paraspinous hemolymphangioma. A 15-year-old girl with an incidental finding of a paraspinous hemolymphangioma is presented along with her history, physical exam, radiographic findings, and operative course. The possible pathogenesis, treatment approach, and clinical dilemmas are also discussed. Given the well-known relationship between tumors and scoliosis, a benign paraspinous vascular and lymphatic tumor may be responsible for the presence of scoliosis in a small number of patients.