Background Although cerebral venous thrombosis/cerebral sinus thrombosis (CVT/CST) remains a relatively uncommon cause of stroke and other neurologic complications, the widespread availability of noninvasive brain imaging has led to an increase in its diagnosis. Purpose Through a review and description of its epidemiology, clinical features and treatment, to heighten awareness of CVT/CST. Methodology Via a systematic review of the more recently published medical literature relevant to the topic and focusing in particular on primary sources, we compiled data related to the incidence of CVT/CST and its diagnosis, treatment and prognosis. Results Thrombosis of a cerebral vein or sinus may occur in individuals of any age and may be caused by a myriad of prothrombotic conditions, both primary and acquired. The clinical presentation of CVT/CST is widely variable, but headache is present in the great majority of cases, and the predominant symptom in many. The headache associated with CVT/CST may be acute, severe, and even “thunderclap” in character, or it may be chronic, pervasive, and of lower intensity. Conclusions Given its eclectic epidemiology, its potential to produce a highly unfavorable clinical outcome, and evidence suggesting that specific treatment improves outcome, CVT/CST is a disorder whose salient features should be familiar to virtually all clinicians.