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A 70-year-old woman was referred to our clinic for evaluation and treatment of a recurring case of adenoid cystic carcinoma of the lacrimal gland. She had been diagnosed with this tumor three years earlier, and a local resection was attempted on two occasions. We encountered this patient on the third recurrence of the tumor, and a thorough work-up revealed extensive local involvement but no metastases. A total exenteration of the right orbit was then performed with aggressive resection of local extension followed by irradiation. Thirteen years later, our patient presented with metastases throughout her scalp. She died shortly thereafter, having survived a total of 16.5 years after the time of her initial treatment and 13 years after exenteration with bony removal of the orbit and irradiation. A review of the literature reveals that this patient had one of the longest reported survival times in the literature from the time of initial treatment. Also noteworthy is the 13 years this patient survived after aggressive surgical resection combined with radiotherapy following two previous incomplete resections.