Background and purpose: Posterior urethral valve (PUV) is one of the most severe urinary tract anomalies presenting as antenatal hydronephrosis that could lead to severe dilatation and functional impairment of one or both kidneys. The aim of this study was to analyze our experience on patients with a diagnosis of PUV. Materials and Methods: In this prospective study neonates with a diagnosis of PUV were enrolled. The patients were assessed clinically for growth and hypertension and other clinical complaints specially voiding dysfunctions. Urinary tract ultrasonography study, functional renal scan, and voiding cystourethrography performed periodically. The patients were assessed for occurrence of hypertension, growth disturbances, long term urinary tract dilatation, persistence of vesicoureteral reflux (VUR), recurrent urinary tract infection (UTI), nephrolithiasis, voiding dysfunction, scar formation chronic renal failure (CRF) and finally death. Results: A total of 24 children with PUV were enrolled, 18 (75%) had VUR. The mean standard deviation score for height to age was −0.10. Hydronephrosis was persisted in 22 (86%) of patients. VUR was resolved in 9 (56%). UTI was found in follow-up of 15 patients, 10 of them had recurrent episodes. Nephrolithiasis was found in 8 (33%), persistent renal damage in 14 (70%) and CRF in 9 (37.5%) of patients, 5 of them had end-stage renal disease. Voiding dysfunction was observed in 5 (36%) of patients, enuresis in 3 (21%) of them. Two patients were died in the course of disease. Conclusion: PUV is an important obstructive process with potential long-term complication with the need to assess the patients for long periods of time.