Osmotic demyelination syndrome (ODS) is a general term that has become commonplace in the practice of medicine, encompassing both central pontine myelinolysis and extrapontine myelinolysis. Historically ODS arises as a serious complication of rapid correction of hyponatremia, yet its manifestations seem to be influenced by a multifactorial process. Further understanding of this rare demyelinating disease has elucidated the significant role of other electrolyte disturbances and the presence of chronic comorbidities as disease risk factors. This review discusses the current research regarding the pathophysiology, clinical manifestations, neuroimaging features, patient management, and prognosis of osmotic demyelination syndrome. We hope that this review will further endorse and aid in the proper diagnosis of ODS and its suitable management through the understanding of clinical and imaging correlations and outcomes, and the comorbid factors that may predispose the development of ODS in certain patient populations.