A 35-year-old woman with a 4-year history of generalized Wegener granulomatosis (WG) had clinically controlled disease. She was evaluated for a 6-month history of right lacrimal sac mass. On examination, a right chronic dacryocystitis and mucocele were observed. A right external dacryocystorhinostomy was performed. The surgical biopsy specimen from the lacrimal sac showed leukocytoclastic vasculitis with more aggressive damage to the small vessels in the deeper mucosa and focal microhemorrhages. The patient was free of symptoms 1 year after surgery. We believe this is the first report of generalized WG presenting features of an active vasculitis of the lacrimal sac wall on surgical biopsy specimen. We conclude that the lacrimal drainage system can be affected directly by focal WG vasculitis, suggesting that nasolacrimal duct obstruction is not always due to contiguous paranasal disease.