Blepharoptosis correction in oculopharyngeal muscular dystrophy (OPMD) patients may result in severe ocular complications owing to lagophthalmos and ophthalmoplegia. Managing the acute episode to prevent further aggravation of the keratopathy or blindness is of paramount importance. A review of the literature for severe chemosis, keratopathy, and corneal ulceration in the patient population was performed using the PubMed database, with key words including ptosis surgery, ptosis correction, ptosis repair, and oculopharyngeal muscular dystrophy. A retrospective review of all patients with blepharoptosis from a single surgeon from September 2009 and May 2017 was performed, selecting those with OPMD who underwent blepharoptosis correction. Our literature review revealed a total of 15 articles after excluding repeated articles and selecting those meeting our inclusion criteria. A total of 232 OPMD patients underwent blepharoptosis correction. Severe ocular complications were noted in 7 patients, with treatment unspecified. For 9 years, 2 OPMD patients at our institute underwent blepharoptosis correction, with one developing severe acute keratitis, chemosis, and corneal ulceration due to lagophthalmos and ophthalmoplegia. Use of the temporary drawstring tarsorrhaphy and topical eye drop treatment for 2 weeks led to resolution of corneal ulcerations without necessitating further intervention. Severe ocular complications may occur after blepharoptosis correction in OPMD patients, potentially owing to lagophthalmos and ophthalmoplegia. Temporary drawstring tarsorrhaphy is an effective option to treat these adverse outcomes.