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A 27-year-old male with nonobstructive hydronephrosis was referred from the urology department for polyuria evaluation andmanagement. The patient was hospitalized for urinary tract infection and cystostomy was performed due to neurogenic bladder ofunknown origin. The patient was of short stature and had visual impairment. From the interview, we discovered he had beensuffering from polyuria and polydipsia for more than 20 years. Urine output was 13 L/day and urine osmolarity was 85 mOsm/kg. The results of a water deprivation test were consistent with central diabetes insipidus. Septo-optic dysplasia (SOD) was observed onbrain magnetic resonance imaging (MRI). SOD is a very rare condition characterized by agenesis of the septum pellucidum orcorpus callosum, which may cause optic nerve aplasia or hypoplasia, midbrain abnormalities and/or hypopituitarism. Afterdesmopressin treatment, polyuria and hydronephrosis were improved. We report a case of a 27-year-old male diagnosed with SODincluding diabetes insipidus, resulting in nonobstructive hydronephrosis.