A 14-year-old boy with bilateral epiphora since birth had absent lacrimal puncta, oral leukoplakia, agenesis of the nails, cutaneous dyschromia, and pancytopenia, findings noted in the rare syndrome of dyskeratosis congenita. Bilateral conjunctivodacryocystorhinostomy was complicated by persistent hemorrhage despite preoperative platelet transfusion. The hemorrhage was controlled by administration of an antifibrinolytic agent, aminocaproic acid. Lacrimal excretory anomalies may be the initial manifestation of a serious systemic disorder, dyskeratosis congenita. Surgical management in this disorder should include appropriate hematologic support.