The release of fat and bone marrow fragments is a common occurrence following traumatic and nontraumatic events. In most cases, they go symptomless or cause only minor disturbances, but occasionally they can determine a multiorgan dysfunction whose severity ranges from mild to fatal. The authors describe the case of a patient who became deeply comatose and ultimately died after a traffic accident in which he suffered the exposed right femoral and tibial fracture in the absence of other injuries. He underwent the external fixation of the fractured bones 2 hours after the admission under general anesthesia. Three hours later, he failed to awake at the suspension of the anesthetic agents and became anisocoric; a CT scan demonstrated a diffuse cerebral edema with the herniation of the cerebellar tonsils; these abnormalities were unresponsive to the treatment and the brain death was one day later. The causes, the mechanisms, the symptoms, the prevention, and the treatment of the syndrome are reviewed and discussed.