Background: Lacrimal sac tumors are very rare and are often missed because patients present with features consistent with chronic dacryocystitis. Squamous cell carcinoma is the common est lacrimal sac malignancy. Although primary mucoepidermoid carcinomas of the lacrimal sac are rare, they are locally aggressive. Furthermore, their proximity to vital structures and the skull base makes them potentially lifethreatening. Multidisciplinary management is required, and wide excision followed by chemoradiation is the recommended treatment. Case: Here, we report a 65yearold male who presented with watering eyes and a mass in the region of the medial canthus. A dia gnosis of primary mucoepidermoid carcinoma of the lacrimal sac was made, and the case was managed successfully with radical surgery and reconstruction. The tumor was resected using the extended LynchHowarth incision and the resulting defect was reconstructed using a forehead flap. Histopathological examination of the excised specimen revealed mucoepidermoid carcinoma. Immunohistochemical analysis revealed that the speci men was positive for epithelial growth factor receptor and Ki67 protein. The patient was refe rred for postoperative chemoradiation. The literature is reviewed and pathological features, including immunohistochemistry are discussed. Conclusion: Primary mucoepidermoid carci noma of the lacrimal sac is a rare, locally aggressive tumor that is often mistaken for dacryocys titis. The treatment of choice is radical surgery followed by chemoradiation.