Our purpose is to report a case of trigeminal neuralgia caused by vertebrobasilar dolichoectasia treated with microvascular decompression. A 63-year-old man sought treatment for a recurrent lancinating left facial pain in V2 and V3 trigeminal territories. The computed tomography angiography revealed a mechanical compression of the left trigeminal nerve due to vertebrobasilar dolichoectasia. The patient was submitted to a left suboccipital craniotomy. Shredded Teflon was introduced in the conflicting neurovascular area, achieving a satisfactory decompression. The patients pain resolved immediately. Vertebrobasilar dolichoectasia is a rare cause of trigeminal neuralgia and a successful outcome can be achieved with microvascular decompression.