© 2015 American Academy of Ophthalmology. Purpose To describe a newly recognized clinical syndrome consisting of ptosis, diplopia, vertical gaze limitation, and abduction weakness that can occur after orbital roof removal during orbito-zygomatic-pterional craniotomy. Design Case series. Participants Eight study patients (7 women), 44 to 80 years of age, with neuro-ophthalmic symptoms after pterional craniotomy. Methods Case description of 8 study patients. Main Outcome Measures Presence of ptosis, diplopia, and gaze limitation. Results Eight patients had neuro-ophthalmic findings after pterional craniotomy for meningioma removal or aneurysm clipping. The cardinal features were ptosis, limited elevation, and hypotropia. Three patients also had limitation of downgaze and 2 patients had limitation of abduction. Imaging showed loss of the fat layers that normally envelop the superior rectus and levator palpebrae superioris. The muscles appeared attached to the defect in the orbital roof. Ptosis and diplopia developed in 2 patients despite Medpor titanium mesh implants. Deficits in all patients showed spontaneous improvement. In 2 patients, a levator advancement was required to repair ptosis. In 3 patients, an inferior rectus recession using an adjustable suture was performed to treat vertical diplopia. Follow-up a mean of 6.5 years later revealed that all patients had a slight residual upgaze deficit, but alignment was orthotropic in primary gaze. Conclusions After pterional craniotomy, ptosis, diplopia, and vertical gaze limitation can result from tethering of the superior rectus-levator palpebrae superioris complex to the surgical defect in the orbital roof. Lateral rectus function sometimes is compromised by muscle attachment to the lateral orbital osteotomy. This syndrome occurs in approximately 1% of patients after removal of the orbital roof and can be treated, if necessary, by prism glasses or surgery.