Since it was initially described, fat embolism syndrome (FES) has remained one of the least clearly understood complications of trauma. This article is a review of the classic and current literature on FES with regard to its causes, pathophysiology, clinical presentation, diagnosis, and treatment. FES is associated with many traumatic and nontraumatic conditions, but is most commonly associated with fractures of long bones of the lower extremity. The pathophysiology is thought to be a cascade of events which can lead to adult respiratory distress syndrome (ARDS). Signs and symptoms of clinical FES usually begin within 24 to 48 hours after trauma. The classic triad involves pulmonary changes, cerebral dysfunction, and petechial rash. Clinical diagnosis is key because laboratory and roentgenographic diagnosis is not specific. Treatment consists of careful initial handling, early stabilization of fractures, careful volume replacement, analgesia, respiratory support, and perhaps steroids. The vast majority of patients today survive FES without sequelae.