A 70-year-old man was referred to our hospital to undergo treatment for hepatocellular carcinoma. In hospital, he complained of hematochezia and a laboratory analysis revealed a decreased level of hemoglobin. Abdominal computed tomography revealed a tumor in the small intestine, with slow enhancement of the dorsal region. Double-balloon enteroscopy revealed a submucosal tumor with a depression in the jejunum. Partial enterectomy was performed and a pathological examination demonstrated the presence of a solitary varix. Solitary varix in the small intestine has not been reported previously. We herein report an extremely rare case of solitary varix in the jejunum.