This update analyzes the episodes of stroke produced by nonarteriosclerotic vasculopathy and cerebral venous thrombosis. This is a group of uncommon processes that have significant clinical importance, given that they frequently cause stroke in young patients and usually have a specific treatment. Nonarteriosclerotic vasculopathies can be divided into groups according to whether they have an inflammatory (e.g., temporal arteritis), infectious (e.g., meningovascular syphilis) or genetic (e.g., cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) etiology or if there is no apparent cause (arterial dissection). Cerebral venous thrombosis can occur at any age and can be caused by a multitude of acquired or genetic diseases and by triggers such as drugs. To a considerable degree, its etiology usually indicates the prognosis of the patient. Approximately 10% of patients will have a poor outcome. Anticoagulants, in both the acute phase and subacute chronic phase, are the treatment of choice.