The treatment of asymptomatic hydronephrosis due to ureteropelvic junction obstruction (UPJO), as well as the timing and indications for surgical intervention, remain controversial. Diabetes insipidus (DI) is a rare disease in infants that is known to cause non-obstructive hydronephrosis, while its association with obstructive hydronephrosis has not been reported. Some studies have found that increased water intake is a predisposing risk factor of developing hydronephrosis. However, there are no studies or guidelines that indicate the specific follow-up intervals and surgical indications for mild hydronephrosis if predisposing risk factors persist. A 46-month-old boy was admitted to our Urology Department with a history of Society of Fetal Urology (SFU) Grade 2 prenatal left hydronephrosis, which was stable at SFU Grade 1-2 at regular postnatal follow-ups. The patient developed polydipsia and polyuria three months prior to admission, then he was considered as primary polydipsia by endocrinology and was treated with fluid restriction while the examination was negative. Renal ultrasound at follow-up demonstrated severe left hydronephrosis with an anterior-posterior diameter (APD) of 6.25 cm three months after symptom onset. Diuretic renography (DR) revealed a renal function of 13.7% with a glomerular filtration rate (GFR) of 11.25 mL/min. The patient was otherwise asymptomatic without any abdominal pain or vomiting. He underwent left pyelostomy immediately as well as laparoscopic left dismembered ureteropelvioplasty after three months, and a diagnosis of UPJO was confirmed. The patient had an uncomplicated postoperative recovery and the result of follow-up renal ultrasound was stable. However, the symptoms of polydipsia and polyuria did not improve significantly. He underwent pituitary magnetic resonance imaging (MRI) and pathological examination, the results of which were consistent with central DI caused by Langerhans cell hyperplasia four months postoperatively. This case indicates the need to pay attention to children with mild hydronephrosis undergoing regular observation and conservative treatment, as a sudden aggravation of the hydronephrosis and a rapid decline of renal function may occur if DI persists.