Our objective is to explore the clinical manifestations, imaging features, and therapy of trigeminal neuralgia (TN) caused by vertebrobasilar dolichoectasia (VBD). Clinical and imaging data of 11 cases with trigeminal neuralgia caused by VBD were retrospectively analyzed, and relevant literatures were reviewed. Of these 11 patients, 8 were male, and 10 suffered from hypertension. Imaging findings revealed that the vertebrobasilar arteries were pathologically enlarged and tortuous. Facial pain disappeared or was relieved after the microvascular decompression (MVD) in all 11 patients; no recrudescence was found with an average of 22-month follow-up. We concluded that TN caused by VBD, a rare clinical disease, mainly occurred in older men with a history of hypertension. CT, MRI, and MRA have great significance in the diagnosis of this disease; MVD is a preferred treatment method.