Sprengel disease (SD) is a congenital condition characterized by upward displacement of the scapula. Deformity may be associated with malformations of other mesodermal structures. SD is usually recognized in childhood. Omovertebral bone may be detected in 25% of SD cases. We present a case an adult woman with multifocal spinal malformations who present with pain and range of motion limitation related to a large omovertebral bone. Computer tomography with 3-dimensional reconstruction was applied in diagnostic process. SD was recognized in the age of 33 years. The patient underwent multiaspect rehabilitation followed by partial excission of the omovertebral bone. We noted favorable functional and cosmetic effect on a follow-up 13 months after surgery. The paper discusses diagnostic and therapeutic issues of SD. Conclusions: Computer tomography with 3D reconstruction is helpful in diagnostics of complex spinal malformations. Problems related to omovertebral bone may be leading complaints of SD. Functional deficit associated with omovertebral bone is an indication to resection of this structure.