Congenital pseudarthrosis of the clavicle is a rare entity of unknown aetiology. Its pathogenesis is related to the embryology of the clavicle. We present a 6-year-old girl with congenital pseudarthrosis of the right clavicle. A prominence was noticed at birth between the middle and distal ends of the clavicle that increased in size when the right shoulder was actively mobilised. Radiographic examination revealed a hypertrophic pseudarthrosis of the clavicle. The pseudarthrosis was resected and the clavicular segments were fixed with an external fixator for 2 months until union. Clinical results were excellent at the 7-year follow-up: the right shoulder was pain-free and the appearance satisfactory. Surgical treatment of congenital pseudarthrosis of the clavicle in children using an external fixator provides a better cosmetic outcome with smaller postoperative scars and avoids a second surgical procedure to remove the implants.