Background: Angiomatoid fibrous histiocytoma (AFH) is a rare type of sarcoma with low-grade malignancy that usually occurs in young subjects. AFH is uncommon in the head and neck region. Methods: We describe an exceptional case of localization in the neck. This is the first report of a rare variant of AFH presenting in a 42-year-old woman. The tumor was situated posterior to the carotid artery adhering to the prevertebral plane and invading the sympathetic cervical chain. In this setting, we decided to perform a surgical exploration with frozen section biopsy. The frozen section revealed a fusiform tumor proliferation resembling a sarcoma. A FISH study concluded on the presence of a EWSR1 22q (22) gene rearrangement. Furthermore, the immunohistochemical study revealed anti-EMA positive cells. The final pathological description concluded on the presence of an AFH, which was excised by surgery alone. Results: After a 2 year follow-up period, the patient is free of disease. Angiomatoid fibrous histiocytoma (AFH) is a rare sarcoma subtype, and mis diagnosis can lead to its over treatment. A precise description of the pathological response and a multidisciplinary discussion can lead to a correct decision. Conclusion: AFH is a tumor with local evolution, and surgery is the mainstay of its management. Complete local excision is recommended to hamper local recurrences.