Acute disseminated encephalomyelitis (ADEM), also known as postinfectious encephalomyelitis, is an immunologically mediated demyelinating disorder affecting the central nervous system that typically occurs after infection or vaccination. The prognosis of ADEM is generally favorable. In a small subset of patients with ADEM, however, fulminant cerebral edema requiring neurosurgical intervention will develop. Few recommendations are available to help the neurosurgeon in dealing with such cases. In this report, the authors present the case of a patient with ADEM in whom central brain herniation developed secondary to medically intractable cerebral edema. The authors review the salient features of the disease and suggest a role for neurosurgeons in cases of fulminant ADEM.