Background: Cerebral metastasis from Adenoid cyst Carcinoma (ACC) remains extremely rare. Case description: 62-year-old female, with a history of thyroidectomy for goiter surgery associated with left cervical lymphadenopathy five years ago. She presented a decrease in visual acuity at 6/10 in the right eye. Brain MRI revealed an intra-conical right orbital lesion process associated with a right frontal cerebral lesion process, three other osteolytic lesions of the cranial vault, lung metastases, and hepatic metastasis. Thyroid ACC with Antibody Anti CD117+ after stereotaxic biopsy of brain lesion was retained by the pathologist. She underwent radiotherapy and chemotherapy management. At 6 months follow-up, the cerebral and liver localisations didn’t progress, unfortunately, the lung mass was progressed from 4 cm to 6 cm. Conclusion: Multiple brain metastasis from ACC of the thyroid is extremely rare. Diagnosis and Management could present some challenges for surgeons and oncologists Keywords: Adenoid cyst carcinoma; Thyroid; Brain metastasis; CD117; Stereotaxic biopsy.