Objective: To investigate the etiological distribution of hydronephrosis caused by upper urinary tract obstruction in adult patients and to improve the diagnostic accuracy for this condition. Methods: The clinical information of adult patients with newly diagnosed hydronephrosis in Upper Urinary Tract Repair Outpatient Clinic of Peking University First Hospital from May 2020 to May 2021 were prospectively and continuously collected. Patients with ureteral calculi or upper urinary tract tumor were excluded. A total of 767 patients were involved. The underlying causes of upper urinary tract obstruction were identified by senior urological surgeons according to symptoms, medical history, physical examination, and a range of diagnostic imaging techniques including ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), retrograde pyelography, antegrade pyelography, radionuclide renogram and ureteroscopy. Results: Among the 767 patients, 359(46.8%) were male and 408(53.2%) were female. The median age of these patients was 37 years (range, 14-84 years). Hydronephrosis was observed at left-sided in 357 cases(46.6%), right-sided in 251 cases(32.7%), and bilateral in 159 cases(20.7%). The causes of hydronephrosis were classified as follows: (1) Non-iatrogenic factors were found in 464 cases (60.5%). These included urinary malformations in 355 cases(76.5%), infection in 29 cases(6.3%), pelvic lipomatosis and/or cystitis glandularis in 23 cases(5.0%), ureteral endometriosis in 18 cases(3.9%), retroperitoneal fibrosis in 15 cases(3.2%), trauma in 7 cases(1.5%) and other non-iatrogenic factors in 12 cases(2.6%). Some of these patients had multiple non-iatrogenic causes. Among the 355 cases with urinary system malformations, 252 cases (71.0%) had ureteropelvic junction obstruction. (2) Iatrogenic ureteral injuries accounted for 210 cases (27.4%), including 112 cases(53.3%) of urological surgical injuries, 51 cases(24.3%) of radiotherapy for malignant tumor related injuries, 34 cases(16.2%) of gynecological and obstetrical surgical injuries, and 13 cases(6.2%) of general surgical injuries. (3) The cause of hydronephrosis remained unknown in 93 cases(12.1%). Conclusion: Hydronephrosis in adults due to upper urinary tract obstruction has a diverse range of causes, with urinary malformations and iatrogenic ureteral injuries being significant contributors. Urological surgeon involved in upper urinary tract reconstruction should be familiar with these potential causes to facilitate accurate diagnosis and effective treatment.