Background: Congenital dislocation of the radial head of the elbow is genetically transmitted in some cases and is often associated with syndromes, such as Nail-Patella syndrome, antecubital pterygium and ulnar dysplasia. The natural history of the condition is that symptoms are relatively benign, with only some limitation of motion and deformity. Treatment either involves early attempts at reconstruction or delayed intervention at skeletal maturity with radial head excision. Case Report: We evaluated the radiographic and functional results of radial head excision and transcapitellar K-wire fixation in the treatment of unilateral left side congenital dislocation of the radial head in 15 year old girl with excellent result at final follow-up. Conclusion: Congenital radial head dislocation should be treated with open reduction and internal fixation with/without radial shortening at an earlier age but if untreated/neglected till adolescence, radial head excision after proper evaluation can give excellent results.