Cerebral venous thrombosis (CVT) is a rare but serious complication of ulcerative colitis (UC) with a high morbidity and mortality rate. CVT is not usually readily recognized and treatment may be delayed, impacting on the prognosis. Here we are reporting 2 cases of CVT occurring during a relapse of ulcerative colitis (UC) with review of literature. The first patient was a 27-year-old woman with chronic UC who presented with headaches during a relapse of chronic UC. She was found to have cerebral venous sinus thrombosis. The second one was a 45-year-old man who presented with seizures and focal neurological symptom during a relapse of chronic UC. He was found to have cerebral venous sinus thrombosis complicated by left temporal infarction. Both of them were treated with therapeutic anticoagulation with good improvement. The presence of neurological signs in a patient with UC mostly during exacerbation period, should alert health professionals about the possibility of a CVT.