A 1-year-old Maltese-Poodle mixed breed dog was referred to the cardiology service because of severe exercise intolerance and daily exercise-induced syncopal episodes. Physical examination revealed no abnormalities. Echocardiography showed intermittent underfilling of the heart. Abdominal ultrasound examination revealed a segmental aplasia of the caudal vena cava between the kidneys and the liver, and azygos continuation of the cava. The azygos vein dorsal to the right kidney showed a severe aneurysm with stasis of blood. Computed tomographic angiography showed that the right crus of the diaphragm was probably responsible for the intermittent compression of the dilated azygos vein, which was thought to have subsequently led to insufficient venous return to the heart. The underfilled ventricles could not produce sufficient cardiac output, which caused the assumed cerebral hypoperfusion due to presumed systemic arterial hypotension. Under general anesthesia a self-expanding nitinol stent was implanted into the azygos vein at the level of the diaphragm. All clinical signs resolved immediately after surgery. The dog remained free from clinical signs (6 months after surgery). This is the first report that describes the successful treatment of this congenital vascular anomaly. Ultrasonography of the caudal vena cava should be performed in dogs with unexplained syncope.