Summary

A 48-year-old man presented with proptosis of the left eye, which on excisional biopsy proved to be primary adenosquamous carcinoma of the lacrimal gland. The lesion was excised radically by a limited frontotemporo-orbitozygomatic approach and any further surgeries, such as exenteration, were avoided in view of the patient's wish to preserve the eye and vision. Long-term follow-up is planned to look for any metastasis or recurrence of the tumour. Primary adenosquamous carcinoma of the lacrimal gland is a very rare entity and, following a thorough literature review, only two cases of lacrimal gland adenosquamous carcinoma have been reported so far.