Summary

A 47-year-old man with a history of recurrent pleomorphic adenoma of the right lacrimal gland presented with rapid onset of a swelling in the right orbit. Initial imaging with CT showed that the swelling was grossly involving the extraocular muscles. Hence we had a suspicion of malignant transformation, so a radical approach in the form of right orbital exenteration with anterior skull base resection and temporalis muscle reconstruction was taken. Postoperative histology revealed epithelial–myoepithelial carcinoma, with immunopositivity for epithelial and myoepithelial components. Adjuvant radiation of 60 Gy was given with three-dimensional CT-based planning. This case portrays the importance of adjuvant treatment in recurrent pleomorphic adenoma and chance of malignant transformation in rare histologies.