Angiomatoid fibrous histiocytoma is a rare soft tissue tumor of uncertain differentiation and low metastatic potential, which occurs predominantly in children and young adults. It occurs mostly within the extremities, trunk, head and neck. It can be associated with systemic manifestations such as anemia, pyrexia and malaise. Its morphology is distinct, with an outer shell of lymphoid tissue, sheets of dendritic-like tumor cells with bland nuclei and blood-filled cystic cavities. Herein, we present a case of angiomatoid fibrous histiocytoma with systemic symptoms before any mass was clinically detectable, arising in the scalp of a 10-year-old girl.