The purpose of this study is to report a case of fluid-ventilated scleral rigid gas permeable lens use in the management of ocular cicatricial pemphigoid.A 74-year-old woman was referred to our clinic in May 2007 with a 4-year history of ocular pain and declining visual acuity. She had been diagnosed with ocular cicatricial pemphigoid by her previous eye care provider. Despite aggressive systemic and topical therapy, she developed persistent corneal epithelial defects in both eyes (3 mm in diameter OD, 4 mm x 8 mm OS). Her best spectacle-corrected visual acuity was significantly reduced (20/150 OD, hand motion OS). She was fit with Jupiter scleral lenses for continuous wear to help preserve corneal integrity and visual function.Within 6 weeks of initiation of scleral lens wear, her corneal epithelial defects had healed and progression of stromal thinning was arrested. Her visual acuity with the scleral lenses was 20/50 OD and hand motion in the left eye. Her ocular surface and visual function have been maintained for 17 months after the initiation of scleral lens wear.If severe ocular cicatricial pemphigoid threatens corneal integrity despite traditional management, scleral contact lenses may play a role in the visual rehabilitation and ocular surface protection in select patients with this disease.