Adenoid cystic carcinoma (ACC) of the lacrimal gland is a rare disease, but it is the most common malignant epithelial tumor of the lacrimal gland, with poor prognosis. ACC of the lacrimal gland is notorious for its slow growth and tendency to recur despite surgical therapy and following radiotherapy and/or chemotherapy. Perineural invasion is considered an indicator of poor prognosis and data showed an inherent risk of spread to local invasion and recurrence, especially to skull base. This tumor type occasionally metastasize via haematogenous spread to lungs, brain and bone in decreasing order of frequency. When it happen, metastases are always multiple and wide. We report a case of a 29-year-old woman followed in our centre with right eye ACC of lacrimal gland resected on January 2009 and treated with intraoperatory cyber knife radiotherapy (16 Gy total dose). Histologic report evidenced no signs of perineural invasion and low risk local and distant recurrence. After five years of follow-up spine MRI reported a single lesion in first dorsal vertebrae with neurologic compressive sympthoms, subjected to D1 laminectomy and subsequent radiotherapy. Histology confirmed metastasis of ACC. No radiologic evidence of other locoregional, synchronous or metachronous distant metastasis were described. After treatment, patient is actually in close follow up. In literature case with distal recurrence after many years are described, but always with many metastases in bone, lung and node. At our knowledge, this case report for first time a single metastatic bone site treated with surgery and radiotherapy.