Developmental venous anomalies (DVAs) are benign embryologic vascular variants, and before the advent of computed tomography and magnetic resonance imaging were supposed to be rare conditions. Usually, DVAs are asymptomatic and accidentally discovered during routine brain imaging studies, but sometimes they can be the cause of disabling neurologic symptoms. We describe the clinical and neuroradiologic follow-up of a 62-year-old man with a history of inflammatory bowel disease (IBD) presenting with new onset epilepsy and intracranial hemorrhage caused by thrombosis of a DVA who fully recovered after treatment with oral anticoagulant therapy. Patients with IBD have an increased risk of thrombosis because of inflammatory activity and the hypercoagulable state. Here we describe the first case of DVA thrombosis in a patient with IBD, and we show clinical and neuroradiologic follow-up after anticoagulant therapy.