PURPOSE: To report the efficacy of topical cyclosporine A (CsA) 0.05% drops (Restasis; Allergan, Irvine, CA) in the treatment of superior limbic keratoconjunctivitis (SLK). METHODS: Seven patients with SLK refractory to corticosteroid drops and topical silver nitrate application were treated with 0.05% topical CsA 4 times a day, preservative-free artificial tears, and a lubricant ointment. The patients were evaluated for symptomatic and clinical improvement after 2 weeks, 1 month, 3 months, 6 months, and in 6 patients, >12 months. RESULTS: After 2 weeks of topical CsA treatment, symptoms of foreign-body sensation, irritation, and photophobia and signs of conjunctival hyperemia, tarsal papillary reaction, and filamentous keratopathy improved remarkably in all patients. The patients had some burning of short duration after administration of topical CsA. However, none of them suffered from systemic or serious local side effects. After cessation of the topical CsA treatment, 6 patients were followed up for at least 6 months. In this period, the patients continued using lubricant agents. Three patients (50%) showed signs of recurrence after 3 months, and topical CsA was restarted. The clinical findings improved again within a 2-week period, and none of these patients needed further use of topical steroids. CONCLUSIONS: Topical CsA 0.05% can be effective in patients with SLK who do not respond to other treatment modalities. It can also be used in the long term to prevent recurrences during treatment and to avoid steroid dependency and the side effects of steroids. © 2008 Lippincott Williams & Wilkins, Inc.