Bleeding caused by ectopic varices is an uncommon but important and life-threatening complication of portal hypertension. Spontaneous external hemorrhage from a recanalized umbilical varix is an extremely rare source of ectopic variceal bleeding. We report on the acute presentation of a patient with a bleeding umbilical varix and successful treatment with percutaneous umbilical vein embolization with sclerotherapy. This previously unreported technique is described, which adds a new therapeutic option for the management of this challenging clinical dilemma. A discussion on the recognition and management of ectopic varices is also provided.