Understanding of ocular cicatricial pemphigoid (OCP) has changed dramatically over the last three decades. Progress in basic science research in mucous membrane pemphigoid has been instrumental in outlining the key immune processes that participate in the autoimmune response. Immunomodulatory therapy has provided an avenue for preserving vision and preventing potentially fatal consequences of systemic pemphigoid. In this chapter we focus attention on the immunologic basis for disease and treatment strategies. We propose an algorithmic approach to diagnosis and management of OCP.