Dolichoectasia is a dilatation and elongation of the arteries, usually affecting intracranial basilar and vertebral arteries. It may cause posterior circulation stroke or transient ischemic attacks independent of atherosclerotic disease. Compression of cranial nerves or brainstem leads to hemifacial spasm, trigeminal neuralgia, or brainstem dysfunction (temporary or permanent). Intracranial bleeding is another possible manifestation. In general, the mortality and morbidity is higher in patients with dolichoectasia. Progression of arterial dilatation or elongation is an ominous sign for poor prognosis. Optimal treatment for vertebrobasilar dolichoectasia is uncertain. Adequate control of blood pressure may prevent ischemic or hemorrhagic stroke. In case of ischemic stroke, antiplatelet treatment is indicated for the prevention of recurrent stroke. However, the risk of hemorrhagic stroke should be considered in patients with severe forms of dolichoectasia. Close observation and follow-up neuroimaging is recommended to monitor the progression of the disease and appearance of new ischemic or hemorrhagic lesions. Surgical decompression may be reserved for the medically intractable patients with trigeminal neuralgia or hemifacial spasm.