Central nervous system (CNS) inflammatory demyelinating diseases are a group of disorders that include multiple sclerosis, acute disseminated encephalomyelitis, and neuromyelitis optica. These conditions may result in emergencies because of severe inflammatory destruction of CNS tissues or complications thereof. Most of these conditions are responsive to appropriate therapy and early diagnosis and treatment leads to better outcomes. We discuss the spectrum of emergencies associated with these disorders, as well as clinical features, investigations, and management.