A two‐year‐old, neutered male Bernese Mountain Dog was evaluated for chronic vomiting. CT imaging revealed left hydronephrosis and hydroureter secondary to ureteral constriction due to congenital circumcaval ureter and concomitant levoposition of the caudal vena cava (CVC). Nephroureterectomy was performed, and clinical signs were fully resolved. Although congenital circumcaval ureter has been described significantly in cats, only two cases in dogs were previously reported. Its clinical relevance depends upon the grade of luminal stenosis. This report offers details of the first fully described case with clinical signs, imaging findings, treatment and follow‐up, including histopathology, of a severe pyelectasia caused by entrapment of the ureter by the transposition of the CVC in a dog.