Background: Apraxia of eyelid opening (AEO) primarily has been described as bilateral loss of volitional ability to open the eyes at certain times and often associated with neurodegenerative disease. Rarely, it can occur in isolation and as an idiopathic phenomenon. There are a few reports of unilateral AEO only on awakening from sleep. We report an additional 11 patients with this unusual variation of AEO. Methods: Retrospective, observational case series of patients collected from 3 separate neuro-ophthalmology practices. Results: All 11 patients were Caucasian women with a mean age of 59 years (range 35-80 years). All experienced AEO on awakening from sleep. Eight patients had unilateral AEO, and 3 had bilateral symptoms. The duration of episodes ranged from 3 weeks to several years. Ten of the patients reported manually elevating the eyelid to open it, while 1 patient waited for the eyelid to open spontaneously. After initial manual elevation, all patients reported normal function and position of the eyelids for the remainder of the day. Seven patients had a history of autoimmune disease. Slitlamp and fundus examinations were negative for ocular pathology to explain the patients' symptoms, and 9 patients had unremarkable brain imaging. Conclusions: AEO occurring only on awakening from sleep is a rare entity. Neuroimaging and extensive laboratory testing are not indicated without associated neurologic or ocular findings. There may be a Caucasian female preponderance and autoimmune link in patients with AEO, but further studies are required.