A 48-year-old woman presented with a 5-month history of unilateral epiphora and progressive growth of a left-sided medial canthus mass. CT revealed a nonenhancing soft tissue mass of the left lacrimal sac with widening and erosion of the nasolacrimal canal. The tumor was resected and pathologically confirmed to be malignant melanoma. Despite wide resection and adjuvant radiotherapy, the patient developed metastases to the chest wall. The patient currently is receiving combined immunotherapy and chemotherapy. The clinical course and treatment strategies are reviewed for this highly aggressive and rare neoplasm.