Three categories of congenital elbow dislocations are described in the literature: humeroulnar, humeroradial, and dislocation or joint laxity with other congenital anomalies. Congenital humeroulnar dislocation is a well-defined problem that occurs predominantly in small breeds of dogs and causes severe limb deformity and dysfunction. Early reduction and stabilization are simple to achieve and have provided good results. Radial head dislocations occur most often as a result of growth disturbance of the radius and ulna. There is a lack of conclusive information on congenital radial head dislocations. Regardless of the etiology, radial head dislocation causes rather mild deformity and lameness and can be treated by conservative management, surgical correction, or radial head ostectomy. Dislocation of the elbow may occur concurrently with other rare congenital anomalies and has been described with ectrodactyly and polyarthrodysplasia.