Myxofibrosarcoma was originally described as the myxoid variant of malignant fibrous histiocytoma (MFH). It is uncommon in the head and neck region. We hereby report a case of myxofibrosarcoma in the sphenoid sinuses. The diagnostic and management difficulties are discussed. Close collaboration between surgeon, radiologist, histopathologist and clinical oncologist in makng accurate diagnosis and appropriate management of this rare tumour are emphasized.