Cerebral venous thrombosis is a rare serious neurological disorder that may cause vital or morbid consequences if not diagnosed and treated promptly. It is a unique cerebrovascular disorder that predominantly affects adults in their third and fourth decades. The incidence of CVST in adults is estimated to be 4 cases per million of the population, and 7 cases per million in children. In the last 2 decades, the awareness and prognosis of the disease have improved due to development of sophisticated neuroimaging techniques and effective treatment. This comprehensive review of the topic aims to improve knowledge among neurologists and internists who are involved in the management of these patients.