Headache is the most frequent and often the earliest symptom of cerebral vein and sinus thrombosis (CVT). Intracranial hypertension, vein distension and venous infarction alone or in combination are thought to be the principal mechanisms in the pathophysiology of CVT headache. The absence of specific features and the possibility of presentation without associated neurological signs can make its recognition difficult. The early diagnosis of an association of headache with CVT is crucial for patient well being. Physicians must always consider the possibility of CVT in patients with recent headache, even in the absence of neurological signs or a negative brain CT that seem to rule it out. History of cancer, recent head injury, recurrent venous thrombosis, autoimmune diseases, puerperium and/or pregnancy and the use of oral contraceptives should all raise the attention of the physician for a possible CVT.