A 64-year-old woman was transported suffering from persistent lower abdominal pain, vomiting, and low-grade fever. Magnetic resonance imaging revealed an empty sella (ES) and hormone tests revealed a disappearance of diurnal variation of cortisol, low cortisol and adrenocorticotropic hormone (ACTH) secretion especially in the morning, and poor ACTH-cortisol axis reaction, as well as normal hypothalamus-pituitary gland-thyroid or adrenal gland axis hormone reaction. The cause of ES remained unclear; however, based on a diagnosis as adrenal insufficiency due to inappropriate ACTH secretion caused by total primary ES syndrome, we started hydrocortisone (15 mg/ day). Afterwards, she immediately became symptom-free and was discharged.