Abstract
One rare and low-grade soft tissue tumor with intermediate malignant potential is angiomatoid fibrous histiocytoma (AFH)و which occurs mainly in children and adolescents. The tumor naturally tends to local recurrence and recurrent hemorrhage but rarely to remote metastasis. AFH has been reported in different organs; however, there are rare reports of primary intracranial AFH. The diagnosis of AFH may be difficult due to its occurrence at multiple unusual anatomic sites and its spectrum of morphologic patterns; thus, it is especially important to diagnose it correctly because of the small risk of metastasis and death. The lesion is simply confused with a hematoma, soft tissue hemangioma, or malignant fibrous histiocytoma from clinical and radiographical aspects. We report a case of intracranial AFH in a 5-year-old boy. The tumor is a heterogeneous intra-axial with a size of 78*73mm at the right front temporal. There was also an extra-axial mass measured 8*12mm at the left superior frontal lobe in favor of metastasis. The diagnosis was confirmed using radiographical, immunohistochemical, and molecular tests. AFH is a rare tumor with a high probability of misdiagnosis. Surgeons must be aware of the presence of AFH and conduct a careful follow-up.