Cerebral venous thrombosis (CVT) is an important cause of stroke in young adults. Data from large international registries published in the past two decades have greatly improved our knowledge about the epidemiology, clinical manifestations and prognosis of CVT. The presentation of symptoms is highly variable in this disease, and can range from a patient seen at the clinic with a 1-month history of headache, to a comatose patient admitted to the emergency room. Consequently, the diagnosis of CVT is often delayed or overlooked. A variety of therapies for CVT are available, and each should be used in the appropriate setting, preferably guided by data from randomized trials and well-designed cohort studies. Although deaths from CVT have decreased in the past few decades, mortality remains ∼5-10%. In this Review, we provide a comprehensive and contemporary overview of CVT in adults, with emphasis on advancements made in the past decade on the epidemiology and treatment of this multifaceted condition.