A case of a partial atrial canal defect is described in a nine-month-old female English setter. The patient had a large ostium primum atrial septal defect and a concurrent malformation of the mitral valve. Electrocardiographic and radiographic findings were suggestive of marked enlargement of the right heart and pulmonary overcirculation. Definitive diagnosis and assessment of the haemodynamic consequences were made using echocardiography. The magnitude of the left-to-right intracardiac shunt was estimated by measuring the pulmonary to systemic flow ratio (Qp/Qs) from Doppler-derived pulmonary and aortic blood flow. The results of this report suggest that dogs with a partial atrioventricular canal defect and concurrent mild mitral regurgitation may exhibit no clinical signs during the first years of life, even in cases with a Qp/Qs ratio of greater than 2.