Background Cerebral sinus vein thrombosis (CVT) is a rare but serious complication associated with ulcerative colitis (UC), an idiopathic autoimmune inflammatory disease of the gastrointestinal tract. Management approaches for CVT remain unclear but may include anticoagulation and surgical thrombectomy. Herein, we report a case of a 23-year-old male who developed CVT with a history of UC. The patient was presented to Howard University Hospital when he slipped and fell. On arrival at the hospital, he complained of a headache with an aching sensation, associated with light/sound sensitivity. The patient had a history of uncontrolled UC. He had positive bloody diarrhea, lower abdominal pain, but denied any other neurological deficit. Computed tomography of the head showed left frontoparietal lobe hypodensities. Neurological exam was nonfocal. Vital signs were within normal range, but the patient experienced some memory loss and personality changes. Subsequent diagnosis of CVT was made with magnetic resonance angiography and magnetic resonance venography. Immediate treatment with low-molecular-weight intravenous heparin (18 IU/kg) was introduced. His UC was managed with methylprednisolone (60 mg IV daily), proton pump inhibitors, mesalamine, ciprofloxacin, and metronidazole. His condition gradually improved. On discharge, he was prescribed prednisone, azathioprine for his UC, levetiracetam for seizure, and warfarin with an INR goal of 2-3. In conclusion, the sudden onset and/or acute worsening of neurological status such as headache and mental confusion in a patient with UC should alert the treating physician about the possibility of CVT so that timely intervention could be employed to prevent disabling and potentially lethal sequelae of this disease.