Small bowel varices are usually associated with portal hypertension. Ectopic varices caused by isolated chronic superior mesenteric vein thrombosis are exceedingly rare. Gastrointestinal bleeding secondary to these represents a diagnostic challenge, given the low prevalence and clinical suspicion, and therapeutic dilemma due to the limited scientific evidence available regarding treatment options. We present the case of a 56-year-old man with gastrointestinal bleeding from jejunal varices due to chronic superior mesenteric vein thrombosis, associated with thrombophilia; diagnosed by video capsule endoscopic (VCE) and computed tomography enterography (CTE), with no possibility of endoscopic or radiological management given the extensive involvement, who underwent successful surgery.