Congenital high scapula, more commonly referred to as Sprengel's deformity, was first described by Eulenberg in 1863. Usually other congenital anomalies are associated such as absent or fused ribs, chest wall asymmetry, Klippel-Feil syndrome, cervical ribs, congenital scoliosis, cervical spina biffida was treated with the excision of the elongated acromion and the resection of prominent elongated acromion to be described is an extremely rare malformation. We experienced one case of congenital high scapula associated with anomaly of the acromion, which was treated with the excision of the elongated acromion and the resection of prominent supermedial border of the scapula for the purpose of the cosmetic and functional correction. After 1 year follow up, abduction was improved by only 10 degrees in the glenohumeral joint with the definite cosmetic improvement.