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A 64-year-old man presented with a 3-month history of recurrent conjunctivitis. He was evaluated by an ophthalmologist and submitted to a CT scan that revealed an intraconic mass with invasion of the lateral orbital wall. He was operated, the mass was completely removed (with preservation of the intraorbital structures) and the lateral orbital wall rebuilt. The histopathological analysis revealed an adenoid cystic carcinoma of the lacrimal gland. 4 months later a painful recurrence of the lesion was diagnosed with invasion of the orbital roof and eyelids. After a multidisciplinary discussion and request from the patient, an exenteration of the orbit and removal of the lateral and superior orbital wall and dura mater was performed with the objective of a total resection. The wound and orbit were closed with a vertical rectus abdominis myocutaneous free flap to ensure closure.

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