Case:We describe an adult patient with bilateral Sprengel deformity (BSD) with bilateral omovertebral bone and thoracic spinal failure of segmentation (T2-T4) successfully surgically treated only at 19-year-old. After resection of the bilateral omovertebral bone and simultaneous relocation of the scapula, disabilities of the arm, shoulder, and hand score improved from 26.7 preoperatively to 2.5 at the 3-year follow-up.Conclusion:The surgical treatment of Sprengel deformity is typically recommended between 3 and 7 years of age when significant shoulder impairment and/or severe cosmetic concerns are present. The authors were able to achieve a successful surgical outcome in a patient with BSD despite an unusual late presentation.