CP is a systemic disease of presumed autoimmune etiology that may involve the skin, conjunctiva, or any mucosal surface. As many patients present with ocular symptoms, ophthalmologists must inquire about signs of potential life-threatening esophageal and laryngeal involvement. Ocular involvement is a bilateral chronic cicatricial conjunctivitis that may result in fornix foreshortening, symblepharon formation, trichiasis, and entropion and keratinization of the eyelids and conjunctiva. These mechanical factors are responsible for progressive scarring and vascularization of the ocular surface. Successful systemic treatment of the autoimmune disease is now available; however, the condition often is not diagnosed until significant structural damage to the ocular adnexa has occurred. Our ability to rehabilitate visually patients with advanced disease by penetrating keratoplasty and associated techniques remains extremely limited. Hence, the focus of treatment in OCP should be on early detection of the disease, including conjunctival biopsy, prompt systemic treatment to prevent disease progression, and early aggressive treatment of secondary eyelid and conjunctival abnormalities to avoid the blinding keratopathy so prevalent in this disease.