A case of metastasizing adenocarcinoma of the lacrimal gland with sebaceous differentiation is described, which probably originated in a benign mixed tumour (BMT), with infiltration of the orbital muscles, the bony orbital wall, the neck lymph nodes, and the parotid gland. The patient was a 69-year-old male with a proptosis that had persisted for 31 years. An orbital exenteration was performed. Death occurred due to cerebral tumour infiltration. In view of the sebaceous pattern meibomian gland carcinoma has to be ruled out in the differential diagnosis.