There is a well-known risk of thrombosis in patients with inflammatory bowel disease. Documented cases of intracranial sinus thrombosis in this setting are rare. We present the case of a 30-year-old man with Crohn disease who spontaneously developed a superior sagittal sinus thrombosis and bifrontal infarcts that resulted in death. The patient was heterozygous for factor V Leiden mutation. The literature was searched to assess the frequency of cerebral venous infarcts in inflammatory bowel disease and the role that factor V Leiden plays in thrombosis in such patients.