To describe a case of mucous membrane pemphigoid (MMP) with ocular manifestations in a patient subsequently diagnosed with B-cell chronic lymphocytic leukemia (CLL). Case report and literature review. A 64-year-old man presented with redness and irritation of both eyes. The patient also had blistering lesions on the roof of his mouth, trunk, and buttocks as well as bilateral conjunctival ulceration and symblepharon formation. A complete blood count performed before initiating treatment of presumed mucous membrane pemphigoid revealed a markedly increased white blood cell count (83,920) with a predominance of lymphocytes. Hematologic evaluation including immunophenotyping flow cytometry resulted in the diagnosis of B-cell chronic lymphocytic leukemia. Cyclophosphamide treatment improved his MMP and decreased the white blood cell count. Mucous membrane pemphigoid has been reported in association with a variety of nonhematologic malignancies. This case suggests that mucous membrane pemphigoid may also occur in B-cell CLL and supports the concept that autoantibodies play a role in the pathogenesis of this cicatricial conjunctival disease.