Isolated right varicocele is a rare condition. It could be secondary to a retroperitoneal neoplastic mass involving the right internal spermatic vein, but sometimes, an anatomic variant must be considered. We present a case of a young man with situs inversus and right varicocele, a mirror image of the normal condition, associated with inferior vena cava malformation, who was treated successfully with retrograde selective percutaneous sclerotherapy of the right internal spermatic vein. Selective sclerotherapy of the internal spermatic vein at the time of venography has proved to be a valuable therapeutic option in right-sided varicocele associated with anatomic abnormalities.