Myoepithelioma of the lacrimal gland is an extremely rare monomorphic adenoma, with only six previously reported cases in the literature. We herein report the radiological, clinical and histopathological evaluation of a 46-year-old patient with a history of right eye proptosis and progressive diplopia. A mass in the right lacrimal fossa was detected at MRI and turned out to be benign myoepithelioma of the lacrimal gland at the histopathological examination. The patient underwent excision of the mass by means of a coronal bitemporal approach and lateral orbitotomy. The lesion was entirely removed "en-bloc" with the lacrimal gland. MRI did not show any evidence of recurrent disease 12 months after surgery. The patient was clinically free of disease after 22 months. Surgery is the treatment of choice for this kind of lesion. The coronal bi-temporal approach herein reported offered good exposure of the entire lesion and a satisfactory aesthetic result. Periodic postoperative radiological evaluations with MRI are mandatory.