【Objective】 To investigate the urinary tract characteristics of diabetes insipidus (DI) complicated with upper urinary tract dilatation (UUTD), and to summarize the treatment experience. 【Methods】 The clinical data of 28 DI patients treated in China Rehabilitation Research Center were retrospectively analyzed with UUTD and all urinary tract dysfunction (AUTD) systems to evaluate the urinary tract characteristics. The relevant laboratory results, video-urodynamic recordings (VUDS), UUTD, neurophysiologic tests, treatment regimens and follow-up data were summarized. 【Results】 There were 21 DI cases (75.0%) and 7 cases of DI with neurogenic bladder (NB). Polyuria, polydipsia, urine specific gravity, urine osmotic pressure and water deprivation vasopressin test had diagnostic value for DI. In addition, detailed history, neurological examination, VUDS and neurophysiologic tests had significant diagnostic value for DI with NB. Enterocystoplasty was recommended for 2 DI with NB patients with poor bladder capacity, compliance and renal impairment. For the remaining 26 patients, individualized medication combined with bladder neck incision and appropriate bladder management, including intermittent catheterization, catheter indwelling and regular voiding, achieved satisfactory results. High serum creatinine decreased from (269.8±105.7)μmol/L to (164.4±90.2)μmol/L in 13 patients with abnormal renal function. Forty-eight dilated ureters showed significant improvement in the UUTD grade, and the median grade decreased from 3 to 2. 【Conclusion】 Bladder distension, trabeculation and decreased or absent sensations were common features for DI patients with UUTD. Individualized therapy by medication combined with appropriate bladder management can improve the dilatation and renal function.