We herein report the case of a patient who presented with an acute decrease of visual acuity, hypertension, focal seizures and transient mental dysfunction while undergoing desmopressin treatment. Neuroimaging revealed bilateral occipital-parietal lesions that presented with vasogenic edema. After controlling the hypertension and discontinuing the desmopressin treatment, the patient's condition improved. A follow-up imaging examination performed six months later showed complete resolution of the lesions. It is important to recognize posterior reversible encephalopathy syndrome (PRES) as a rare and serious complication of desmopressin administration. Both the blood pressure and water electrolyte balance should be carefully monitored in patients receiving desmopressin therapy.