Primary adenocarcinomas of the lacrimal gland rarely display sebaceous differentiation. An anaplastic lacrimal gland neoplasm manifesting this feature was excised from a 64-year-old man who initially appeared with recurrent painless subconjunctival hemorrhages. Globe displacement and tumor-induced hyperopia were later findings. Electron microscopic studies performed on the widely excised tumor documented prominent lipid vacuoles, desmosomes. scattered cytoplasmic filaments, and linear segments of basement membrane material. The patient refused radical surgery and instead opted for 6,000 rads of adjuvant radiotherapy. Eleven months postoperatively liver and bone metastases developed, but he was free of local orbital recurrence. Twenty-two months postoperatively he died from complications of his metastatic disease. This most unusual lacrimal neoplasm is discussed in terms of its initial signs and symptoms and its histopathology. When the current case is analyzed in combination with three earlier reports, there is evidence that primary lacrimal gland sebaceous carcinoma is probably among the most malignant orbital tumors due to its uniform early fatality.