Background & Objective: Posterior Urethral Valves (PUV) are common cause of congenital obstructive uropathy in boys and may be associated with urinary tract infection(UTI) and chronic kidney disease(CKD) if not managed timely. The objective of our study was to determine the clinical profile of children with PUV. Methods: This is a descriptive case series comprising of 30 children aged 1-5 years, diagnosed and managed as PUV over six months, conducted at the Department of Pediatric Nephrology, National Institute of Child Health, Karachi. Patients were followed for 12 weeks and the outcome was assessed in terms of recovery, UTI, urinary incontinence and CKD. Descriptive statics were used for data analysis. Results: Thirty cases of PUV were managed during study period. Clinical presentations were poor urinary stream (83%), fever (73%), signs and symptom suggesting UTI (96.6%), pallor (73.3%), acute kidney injury (37%)and urinary retention (13%). UTI was confirmed in 73.3 % and E.Coli was the most common pathogen. Ultrasonography showed bilateral hydronephrosis/hydroureter in 80% and micturating cystourethrogram demonstrated vesicoureteral reflux in 86.66% cases. All patients received intravenous hydration (97%), urinary decompression, and antibiotics. Meropenem was the most commonly used. Packed cell transfusion and peritoneal dialysis was done in 73.33% and 13.3% respectively. Cystoscopic valve fulguration was done in 86.66% and vesicostomy in 13.3%. On short-term follow-up, 60% recovered,16.66% experienced UTI and remained incontinent whereas 23.33% had CKD. Conclusion: Our study showed a high frequency of UTI and AKI. E. coli was most common pathogen. Despite valve fulgration, significant patients had CKD. doi: https://doi.org/10.12669/pjms.38.7.5823 How to cite this:Yamin R, Moorani K, Shaikh M, Yamin S. Clinical profile of children with posterior urethral valve at Tertiary Care Center. Pak J Med Sci. 2022;38(7):1821-1826. doi: https://doi.org/10.12669/pjms.38.7.5823 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.