BACKGROUND: Neurogenic pulmonary edema may be a complication of multiple neurological processes. Although there is debate regarding the underlying pathophysiology, the recognition of neurogenic pulmonary edema is vitally important because of the high-potential for mortality and need for treatment of the underlying disorder. METHODS: We present an example of recurrent neurogenic pulmonary edema in an adolescent boy with multiple sclerosis who was diagnosed with pneumonia at the time of initial presentation. We also review the presenting symptoms, physiologic parameters, and imaging findings from published reports of patients with multiple sclerosis presenting with neurogenic pulmonary edema. RESULTS: Although all 11 cases found via literature review presented with respiratory symptoms, cardiac dysfunction was variable, as was the presence of other neurological findings. All but one case had a documented medullary lesion. Corticosteroids were effective in resolving symptoms. Three patients were not treated with corticosteroids, and one of these died (onset of pulmonary edema during sleep). CONCLUSIONS: Awareness of these patients may expedite recognition and treatment of future patients, thus minimizing time to appropriate treatment and reducing mortality.