Congenital dislocation of the radial head is rare, although it is the most common congenital anomaly of the elbow. A concomitant progressive cubitus valgus of the elbow has not previously been described in literature. We describe a case of an 8-year-old girl with an unilateral congenital radial head dislocation with a progressive cubitus valgus of 35°, caused by a prematurely closing physis of the lateral humeral condyle. This might be caused by an increased pressure on the lateral physis by the anteriorly dislocated radial head. As no complaints or limitations were present, treatment was non-operative with clinical observation, with satisfactory results after a follow-up of 18 months. A concomitant progressive cubitus valgus can be present in patients with a congenital radial head dislocation. Non-operative treatment can provide satisfactory results.