When there is a mass in the superior temporal orbit area, a lacrimal gland tumor should be suspected. We report a rare case of orbital lymphatic malformation that was histologically diagnosed in a patient with typical clinical features of the lacrimal gland.

A 55-year-old female with no underlying disease and no ophthalmic history visited our clinic with a right upper eyelid edema associated with an enlarged painless eyelid mass 1 month prior to her visit. The patient stated that she discovered the mass 1 year previously.The palpebral lobe of the lacrimal gland protruded slightly with congestion of the surrounding conjunctiva. Enhanced computed tomography showed a 3 cm well-defined heterogeneous mass in the right lacrimal gland area and several well-defined round calcifications within the mass. Orbital tissue or bone involvement was not observed. The pleomorphic adenoma of the lacrimal gland was the most clinically suspicious, so complete resection of the mass was performed using lateral orbitotomy. Histopathologically, lymphangioma (lymphatic malformation) originating from the lacrimal gland was diagnosed.

Orbital lymphatic malformation can occur in the lacrimal gland. The present case showed that differential diagnosis can reveal the presence of an adult lacrimal gland tumor.