The objective of this study was to describe a case of marked hypoplasia of the right renal vein with drainage into ipsilateral gonadal vein. A 66-year-old man, known for hypertension and previous smoking, underwent an abdominal ultrasound exam, which detected a juxtarenal aortic aneurysm. Computed tomography scan confirmed the presence of the aortic aneurysm; furthermore it showed an abnormal right kidney venous drainage consistent in a dilated and tortuous vein, which originated at the hilar region, heading caudally and joining the right spermatic vein at level of aortic carrefour. A thin vein-located more cranially with mild and late contrast enhancement-was also demonstrated from right kidney hilum to inferior vena cava, probably representing a remnant of the right main renal vein. To our knowledge, this anatomic variant was never reported in the peer-reviewed literature.