Cerebral venous thrombosis (CVT) is a rare form of deep venous thrombosis (DVT), yet a leading cause of stroke in young adults, with an incidence that seems to be increasing in recent years. Risk factors for CVT overlap with those of DVT in other locations, with the addition of local risk factors, such as infections, head trauma and neurosurgery. The clinical presentation is highly variable, hence the diagnosis of CVT may be delayed or overlooked. Early recognition and timely initiation of anticoagulant treatment are of essence, while decompressive surgery can be lifesaving for patients with impending trans-tentorial herniation. Concomitant intracranial hemorrhage is not a contraindication to anticoagulant treatment. Endovascular treatment may be suggested for selected patients who deteriorate despite anticoagulant treatment. In this review we will provide an extensive and contemporary discussion of the incidence, pathophysiology, risk factors, clinical presentation, neuroimaging and management of CVT, in accordance with recent guidelines. Additionally, we will summarize the latest data with regards to direct oral anticoagulants (DOACs) treatment in CVT.