Double outlet right ventricle (DORV) and double outlet left ventricle (DOLV) are congenital conditions where the great arteries (aorta and pulmonary trunk) both arise mostly or completely from the right or left ventricle. The limited body of evidence about this condition in cats and dogs suggests it is very rare. There does not seem to be a predisposition in certain breeds. The condition is diagnosed usually early and life and is usually medically treated. Despite surgery being a viable option in hu- mans, it is rarely performed in animals. Unfortunately, there is still significant mortality regardless of treatment strategy. Additionally, a lot of animals are not treated at all. Further research is necessary to obtain more insights in the epidemiology of the condition and to assess the different treatment strategies. Retrospective studies should be considered, since prospective trials may be unfeasible due to the rarity of the condition. Subsequently, standard treatment protocols could be developed.