The authors report a case of angiomatoid malignant fibrous histiocytoma (AMFH), affecting a 9-year-old girl, with a highly aggressive clinical course. The tumor, noticed by the patient as a painless nodule in the dorsum of her left foot for 12 months, recurred 8 months after initial excision, and despite wide local reexcision, metastasized 4 months later to the liver and lung, where it grew at an alarming rate, to the extent of occupying the entire left hemithorax in a period of 10 weeks and killed the patient 14 months after initial excision. Review of the literature showed that the culminated rates of recurrence, metastasis, and mortality for AMFH were 23.2%, 8.7%, and 4.3%, respectively, indicating that it is definitely a malignant neoplasm with a potentially fatal outcome.