Résumé : A 45-year-old man with a malignant fibrous histiocytoma (MFH) of the left thalamus is reported. This tumor only rarely originates within the brain. Primarily intracerebral MFH usually has a superficial localization and rarely involves the deep cerebral structures. Our patient presented with complex neuropsychological symptoms, including aphasia, hemispatial neglect and acalculia. These findings are further supporting evidence for the role of the thalamus, particularly of its posterior parts, in language, speech and other neuropsychological functions.