Vertebrobasilar dolichoectasia (VBD) is a rare disorder characterized by elongation and tortuosity of the vertebrobasilar arteries. Clinical manifestations can range from ischemic stroke and progressive compression of cranial nerves and brainstem to cerebral hemorrhage and hydrocephalus. Here, we report a case of intermittent diplopia complaint when looking down with a tilted head position for 3 months. His neuro-ophthalmic examination was within normal limits. Further, magnetic resonance and angiographic images revealed a dolichoectatic basilar artery compressing the right lateral brainstem. VBD treatment is controversial; there is still no effective treatment and only complications can be treated. Surgical options may be useful in very limited situations, but advances in stent technology are promising in the treatment of VBD. To our knowledge, no case in the literature describes intermittent trochlear nerve compression by VBD.