We herein report the case of a 68-year-old man receiving hemodialysis who developed severe hypoglycemia. He became unconscious and exhibited a blood glucose level below 10 mg/dL. We ruled out the possibility of other causes; however, severe hypoglycemia was observed even after starting glucose injections. The patient developed pneumonia and finally died. Although we conducted an autopsy, there were no specific findings explaining the severe hypoglycemia. We believe that carnitine deficiency was possibly involved in the severe hypoglycemia observed in this case. Physicians should be aware of the possibility of carnitine deficiency and/or severe hypoglycemia, especially in hemodialysis patients with malnutrition.