Abstract
This is a case report of a patient presenting with a destructive lesion with histologic features of a low grade malignancy in a predominantly myxoid matrix. Various low grade myxoid malignancies were considered in the differential diagnosis of which an overview is presented. A literature review of the ultrastructural findings and possible histogenesis is discussed along with the diagnostic criteria and recent change in the terminology regarding the malignancies previously diagnosed as myxoid malignant fibrous histiocytomas. A final diagnosis of a myxofibrosarcoma was only possible after assessing the immuno-histochemical profile, results of histochemical stains and ultrastructural features of this lesion.