Duodenal variceal bleeding in patients with portal hypertension due to cirrhosis or other causes is uncommon. We report on a case of a 55-year-old male with an ectopic variceal rupture at the distal fourth part of the duodenum who presented with massive hematochezia and shock. Shortly after achievement of hemodynamic stability, due to the limitation of an endoscopic procedure, we initially attempted to find the bleeding focus by abdominal computed tomography, which showed tortuous duodenal varices that drained into the left gonadal vein. He was treated with first-line balloon-occluded retrograde transvenous obliteration (BRTO), resulting in a favorable long-term outcome without rebleeding three years later. This case suggests that BRTO may be a first-line therapeutic option for control of ruptured duodenal varices, especially at a distal location.