A 16-year-old boy presented with complaints of epiphora and bilateral palpably distended lacrimal sacs. He had a history of Crohn disease involving the small and large intestines, and a history of granulomatous cheilitis. Bilateral dacryocystorhinostomies were performed through an external approach and revealed multiple nodular masses in both lacrimal sacs, which on biopsy, contained granulomatous inflammation consistent with extraintestinal Crohn disease. A literature search from 1966 to the present using the PubMed database revealed three previous publications describing nasolacrimal duct obstruction associated with Crohn disease, but these cases did not show pathologic evidence of granulomatous inflammation. To our knowledge, this is the first reported case of extraintestinal Crohn disease with granulomatous inflammation affecting the lacrimal-outflow system.