The presence of an omovertebral bone with Sprengel’s deformity and Klippel-Feil syndrome is a complex congenital anomaly that is not well understood. It most commonly manifests as cosmetic deformity, limited range of motion, and functional disability, although there are reports of the insidious development of cervical myelopathy. In this paper, the authors present the case of a 49-year-old man with acute neurological deficits after a low-energy mechanism of traumatic spinal cord compression, resulting from an impinging omovertebral bone through a traumatic laminar defect. The patient underwent resection of the omovertebral bone, laminectomy decompression of the spinal canal, and anterior stabilization. This case highlights a rarely discussed complication of undiagnosed Sprengel’s deformity and its associated conditions following even low-energy traumatic mechanisms.