Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth syndrome characterized by anterior abdominal wall defects, macroglossia, and gigantism. A variety of other abnormalities have been described, however association with ectopic spleen and left-sided vena cava has not been reported previously. We report ectopic spleen, left-sided vena cava and the other abdominal imaging findings of an adult BWS case who came up to date without any follow-up from the early childhood.