PURPOSE Ocular cicatricial pemphigoid (OCP) can lead to devastating ocular complications without prompt treatment. A number of immunomodulatory agents have been attempted with varying success. The objective of this study was to evaluate the use of rituximab as an adjuvant to immunomodulatory treatments (IMTs) in refractory OCP. METHODS The clinical records of 14 patients with treatment refractory OCP treated with rituximab as monotherapy or in combination with IMTs were retrospectively reviewed, with a focus on demographics, treatments prerituximab and postrituximab, total number of rituximab infusions, response to treatment, and ocular outcomes including staging and best-corrected visual acuity. RESULTS Thirteen patients (92.9%) achieved a complete response with rituximab over a mean period of 4.3 months. The average sustained complete response time in those without relapse was 29.7 months. Five patients relapsed over a mean period of 15 months, 2 of whom were able to regain control over a mean of 2.5 months with additional rituximab treatments. At the final evaluation, rituximab-based therapy improved ocular outcomes in OCP by stabilizing Foster staging and preventing the deterioration of best-corrected visual acuity in 72% of eyes. In total, 90% of eyes with Foster stages 3 or less did not progress. All patients were able to decrease IMT dosage and/or transition to less potent adjuvant treatments. CONCLUSIONS The consideration of rituximab earlier in treatment of OCP as a rescue and/or maintenance therapy could result in an earlier arrest of disease progression, resulting in preservation of patients' vision, and enable tapering of adjuvant IMT.