BACKGROUND: Vertebrobasilar dolichoectasia (VBD) is a rare vasculopathy. The etiology of this disease is unknown. Transient ischemic attacks (TIAs) of vertebrobasilar system refer to a transient (< 24 hours) lowering of blood flow in the posterior circulation of the brain. We present a case of dolichoectasia in the vertebrobasilar artery that presented with TIAs. CASE REPORT: A hypertensive 54-year-old man with true vertigo, nausea, imbalance, dysarthria, dysmetria, horizontal nystagmus, and gait ataxia was referred to Alzahra Hospital, Isfahan, Iran. The symptoms improved in the 1st day, but recurred in the 2nd day, lasting for 6-7 hours. According to clinical manifestations, a diagnosis of TIAs in the vertebrobasilar circulation was made. Imaging studies showed vascular anomaly. The vascular anomaly was considered as the cause of the patient’s symptoms. A medical management was started using antiplatelet and antihypertensive drugs. The patient was referred for a more evaluation for other vascular anomalies. DISCUSSION: Dolichoectasia usually affects vertebral and basilar arteries and simultaneous involvement of carotid arteries is rare seen in only 0.5% of these patients. The usual symptom of dolichoectasia is ischemia and rarely hemorrhages. The most common type of ischemic stroke is lacunar type. Ischemia evolves from embolic that originate from thrombi or plaques in the walls of the ectatic artery. While hemodynamic effects are the most common cause of the presenting signs and symptoms of the anomaly. We report a case of dolichoectasia that presented with TIAs of the verterbrobasilar artery. VBD is a distinct arteriopathy known as stroke risk.