Fat embolism syndrome (FES) is a common clinical entity that can occasionally have significant neurological sequelae. The authors report a case of cerebral fat embolism and FES that required surgical management of intracra nial pressure (ICP). They also discuss the literature as well as the potential need for neurosurgical management of this disease entity in select patients. A 58yearold woman presented with a seizure episode and altered mental status after suffering a right femur fracture. Head CT studies demonstrated hypointense areas consistent with fat globules at the graywhite matter junction predominantly in the right hemisphere. This CT finding is unique in the literature, as other reports have not included imaging performed early enough to capture this finding. Brain MR images obtained 3 days later revealed T2hyperintense areas with restricted diffusion within the same hemisphere, along with midline shift and subfalcine herniation. These findings steered the patient to the operating room for decompressive hemicraniec tomy. A review of the literature from 1980 to 2012 disclosed 54 cases in 38 reports concerning cerebral fat embolism and FES. Analysis of all the cases revealed that 98% of the patients presented with mental status changes, whereas only 22% had focal signs and/or seizures. A good outcome was seen in 57.6% of patients with coma and/or abnormal posturing on presentation and in 90.5% of patients presenting with mild mental status changes, focal deficits, or sei zure. In the majority of cases ICP was managed conservatively with no surgical intervention. One case featured the use of an ICP monitor, while none featured the use of hemicraniectomy.