Neurofibroma is an extremely rare neural tumor of the lacrimal sac. We present a case of neurofibroma of the lacrimal sac in a 45-year-old lady. Based on the history of intermittent epiphora, presence of a mass in the lacrimal sac region, dacryocystogram suggestive of space occupying lesion within the lacrimal sac and computed tomographic image of a tumor lying in the lacrimal fossa region, a tentative diagnosis of lacrimal sac neoplasm was made. Excision of the tumor with repair of the lacrimal sac was performed. Histopathology proved it to be neurofibroma of the lacrimal sac. Until now, only four cases have been reported worldwide. The paucity of reported cases limits our knowledge regarding the natural course, treatment, results and prognosis of this tumor.