To evaluate efficacy of topical rebamipide for superior limbic keratoconjunctivitis (SLK) in patients with thyroid eye disease. Retrospective, observational case series. Thirty-three eyes from 20 thyroid eye disease patients with SLK who received topical rebamipide (Mucosta ophthalmic suspension unit dose 2%; Otsuka Pharmaceutical Co, Ltd; chemical name, (2RS)-2-(4-chlorobenzoylamino)-3-(2-oxo-1, 2-dihydroquinolin-4-yl) propanoic acid) were included. The following items were evaluated before and 4 weeks after the start of treatments: presence or absence of SLK, rose bengal staining score, area and density classification of fluorescein staining, Schirmer test I results (without topical anesthesia), tear film break-up time, Hertel exophthalmometry values, and margin reflex distances 1 and 2. Twenty-eight eyes showed complete disappearance of SLK after treatment (84.8%; P < .001). The other 5 eyes (15.2%) demonstrated significant improvement, but had residual punctate rose bengal staining and fluorescein staining near the superior corneal limbus. All 5 eyes exhibited at least 1 of the following findings: proptosis of more than 17.7 mm and upper or lower eyelid retractions or both. Incidence of upper eyelid retraction was significantly higher in eyes with SLK than in those without SLK at the 4-week follow-up (P = .021). The severity of rose bengal staining and fluorescein staining improved significantly after treatment (P < .001). Although the Schirmer test results remained constant before and after the treatment (P = .212), tear film break-up time increased significantly in the posttherapeutic state (P = .009). No serious adverse events were reported. Topical rebamipide improved SLK in patients with thyroid eye disease, suggesting a first-line treatment in such patients.