BACKGROUND Vertebrobasilar dolichoectasia (VBD) is a complex progressive arterial disease characterized by dilation, elongation, and tortuosity of the vertebral and basilar arteries, and may be congenital or acquired. VBD may lead to progressive compression of the brainstem, cranial nerve abnormalities, and intracranial hemorrhage, but may also be associated with arterial thrombosis, with ischemic stroke as the most common clinical outcome. CASE REPORT Two cases of VBD are presented, both with acute bilateral ophthalmoplegia and cranial nerve palsies, and vertebrobasilar arterial thrombosis that resulted in ischemic stroke. CONCLUSIONS VBD is a complex arterial disease with a variety of clinical manifestation, with bilateral ophthalmoplegia being a rare presentation. Clinical management of VBD is a challenge as there are no current management guidelines. Therefore, clinical management of cases of VBD should be individualized to balance the risks and benefits of treatment options for each patient.