Introduction: A hemangioblastoma is an uncommon central nervous system (CNS) tumor. It is typically slow-growing and sporadic in occurrence, but up to 25% of cases are associated with von Hippel-Lindau disease. Generally found in the cerebellum, brainstem, or in the spinal cord, there are only a few reported cases of hemangiomablastomas outside the CNS, and these appear to be limited to the kidney, colon, and the retroperitoneum. Hemangiomablastomas are highly vascular lesions and in this presentation we summarize a unique case of acute GI bleeding secondary to hemangiomablastoma in the small bowel. Case Report: A 35-year-old Caucasian male with an unremarkable past medical history presented with syncopal episodes that began after having multiple bowel movements with black stools and subsequent hematochezia. He denied previous episodes of GI bleeding or anemia. He had no complaints of abdominal pain, heartburn, dysphasia, or weight loss. He had recently taken amoxicillin for a URI, and he had taken ibuprofen for several days leading up to his hospitalization. He denied sick contacts or recent travel. The family history was unremarkable. The patient's physical exam was essentially benign. Specifically, he had no abdominal tenderness. His laboratory tests included a hemoglobin level of 11.0 g/DL, and a BUN/creatinine of 25/1.2 mg/DL. The CT scan of the abdomen/pelvis found no abnormalities. The patient subsequently underwent an EGD and colonoscopy, which were both unremarkable. A Meckel's scan was negative, with no evidence for focal abnormal radiotracer activity. A video capsule endoscopy was then performed, and an ulcerated lesion was noted in the distal jejunum. The patient was referred to surgery and underwent an uneventful laparoscopic small bowel resection of the lesion. The surgical pathology results identified an extracerebellar hemangioblastoma. This diagnosis was supported by immunohistochemistry that showed that the stromal cells between the capillaries were negative for CD34, yet clearly positive for inhibin. The neooplastic cells also showed cytoplastic brachyuric expression consistent with hemangioblastoma. The patient has since done well, without recurrence of GI bleeding or other negative sequelae. Conclusion: To our knowledge, this appears to be the first reported case of a hemangioblastoma presenting in the small bowel. While extraordinarily rare, a hemangioblastoma should be considered in the differential for small bowel pathology associated with GI bleeding.