A 60-year-old man with no past medical history presented with a 14-month history of a painless medial left lower eyelid mass causing mechanical ectropion and discharge from the inferior punctum. Excisional biopsy was performed via a transconjunctival canaliculotomy and histopathology revealed a dense plasma cell infiltrate with IgG4+ plasma cells >100/high power field and an IgG4/IgG ratio of 65%. Serum IgG4 was found to be elevated at 224 mg/dl (reference range: 4-86 mg/dl). Systemic work up with PET CT was negative. The patient's signs and symptoms all resolved without medical treatment and he remains disease free 24 months following surgery. A single case of IgG4 disease of the nasolacrimal duct has been reported, but to the authors' knowledge, disease involving the proximal nasolacrimal outflow system has yet to be described. The authors herein report a case of IgG4-related disease involving the punctum and canaliculus.