Sprengel deformity (ie, congenital elevation of the scapula) is a rare clinical entity. However, it is the most common congenital anomaly of the shoulder. Sprengel deformity is caused by abnormal descent of the scapula during embryonic development. Sprengel deformity is associated with cosmetic deformity and decreased shoulder function. Diagnostic confusion with limited scoliosis can be dangerous to the patient because it may delay proper treatment of other abnormalities that may be present with even mild cases. Sprengel deformity is commonly linked to a variety of conditions, including Klippel-Feil syndrome, scoliosis, and rib anomalies. Nonsurgical management can be considered for mild cases. Surgical management is typically warranted for more severe cases, with the goal of improving cosmesis and function. Surgical techniques are centered on resection of the protruding portion of the scapula and inferior translation of the scapula. Recent long-term studies indicate that patients treated surgically maintain improved shoulder function and appearance.