BACKGROUND Here we present a rare case of localized amyloidosis involving the nasolacrimal duct and lacrimal sac which was managed by endoscopic surgery.CASE SUMMARY A 50-year-old man whose medical history included bilateral ventricular fold and vocal cord amyloidosis complained of bilateral epiphora.Magnetic resonance imaging revealed a neoplasm within the nasolacrimal sac.Characteristic positivity for Congo red staining and birefringence under a polarized microscope proved the diagnosis of amyloidosis.Dacryocystorhinostomy via an endoscope obtained a favorable result.A one-year follow-up found no recurrence.CONCLUSION There are few reports on amyloidosis involving the lacrimal outflow system,and management and outcome are not clear.Endoscopic dacryocystorhinostomy can be a choice to relieve symptoms.Regular follow-up and monitoring of systemic diseases are highly recommended.