Background Congenital radial head dislocation by far is a rare condition and also the most common congenital anomaly of elbow. It causes symptoms such as joint stiffness, snapping, locking, or pain; however the diagnosis is sometimes made incidentally from X-rays. Surgical management includes osteotomy of radius, osteotomy of ulna, or radial head excision. Aim and objective The treatment goal is to ameliorate the symptoms and obtain a joint with a full range of motion. Case description Only unilateral cases have been described in literature until now. This article presents a case of bilateral congenital radial head dislocation in a pediatric girl with progressing valgus deformity, treated surgically by a newer technique. We successfully performed an ulnar osteotomy and distraction osteogenesis of ulna with an UMEX external fixator. Once the radial head was brought to the level of the joint, open reduction was done. Conclusion The clinical and functional outcomes were satisfactory. Clinical significance Even though various surgical options are available for the management of congenital radial head dislocation, radial head preserving surgery will benefit the patient on the long run by avoiding the development of distal radioulnar joint problems. Our procedure helps in achieving this goal. How to cite this article Ramprasath DR, Esthak AJM. A Rare Case of Bilateral Congenital Radial Head Dislocation: A Case Report. J Orth Joint Surg 2020;2(2):66–69.