High-grade myofibroblastic sarcoma (HGMS) is a rare malignancy characterized by a high rate of recurrence and metastasis. This form of malignancy tends to be indolent in nature and patients’ initial complaints are usually a painless mass. In this report, we present a case of a 40-year-old male who was initially evaluated for a left knee mass. Imaging studies and biopsy evaluation concluded the diagnosis of low grade myofibroblastic sarcoma of the distal femur and surrounding soft tissue. Despite adherence to current treatment standards for this particular malignancy, the patient experienced recurrence requiring further surgical intervention, adjuvant chemotherapy and radiotherapy. Recurrent tumor was in the form of high-grade myofibroblastic sarcoma. After exhausting all available treatment options, the patient expired secondary to multi-organ failure associated with widespread metastases. In this report, we discuss how to differentiate this sarcoma from other types of sarcomas and provide a brief review of the literature.