Osmotic demyelination syndrome (ODS) is a demyelinating disorder of the central nervous system. It usually occurs with rapid correction of severe chronic hyponatremia. ODS is rarely seen as a complication of hyperglycemia. Herein, we report a rare presentation of ODS secondary to hyperosmolar hyperglycemic state. A 28-year-old female with type 1 diabetes, hypertension, seizure disorder, and end-stage renal disease on hemodialysis was brought from a shelter with two days of unresponsiveness and developed ODS after hyperosmolar hyperglycemic state in long-standing uncontrolled diabetes with normal serum electrolyte levels.