Malignant fibrous histiocytoma (MFH) is a morphologically ill-defined tumour of the soft tissues and may involve nearly every organ of the body. MFH of the spermatic cord represents an extremely rare entity and reports of it in the literature are limited. We report a 69-year-old man found to have a left spermatic cord MFH and retroperitoneal and mediastinal lymphadenopathy, who was treated with radical orchiectomy and adjuvant chemotherapy. The morphological findings of the spermatic tumour are presented and the literature is reviewed to clarify the potential diagnostic/therapeutic approaches and the prognosis related to spermatic cord MFH. Copyright © 2006 Via Medica.