The study was to detect the pathogenic bacterial profile and antibiogram among children with congenital anomalies of the kidney and urinary tract (CAKUT). Patients and Methods: A retrospective analysis was performed using medical records of urine culture results and antibiotic susceptibility results in patients with UTIs from March 2017 to March 2022. Antimicrobial susceptibility pattern was determined by a standard agar disc diffusion method. Results: A total of 568 children were included. The proportion of culture-positive UTI was 59.15% (336/568). More than nine types of bacteria were isolated with most pathogens being Gram-negative species. Among Gram-negative isolates, the predominant bacteria were Escherichia coli (30.95%, 104/336) followed by Klebsiella pneumoniae (9.23%). Escherichia coli isolates were highly sensitive to amikacin (95.19%), ertapenem (94.23%), nitrofurantoin (93.27%), imipenem (91.35%), and piperacillin-tazobactam (90.38%) and high rate of resistant were also detected to ampicillin (92.31%), cephazolin (73.08%), ceftriaxone (70.19%), trimethoprimsulfamethoxazole (61.54%) and ampicillin-sulbactam (57.69%). Klebsiella pneumoniae isolates showed sensitive to ertapenem (96.77%), amikacin (96.77%), imipenem (93.55%), piperacillin-tazobactam (90.32%) and gentamicin (83.87%), while highly resistant were observed to ampicillin (96.77%), cephazolin (74.19%), ceftazidime (61.29%), ceftriaxone (61.29%), and aztreonam (61.29%). The isolated Gram-positive bacteria mainly contained Enterococcus faecalis and Enterococcus faecium (each 15.77%). Enterococcus faecalis were sensitive to vancomycin, penicillin-G, tigecycline, nitrofurantoin and linezolid (100%, 94.34%, 88.68%, 88.68%, 86.79, respectively) and resistant to tetracycline (86.79%), quinupristi (83.02%), erythromycin (73.58%). Enterococcus faecium also showed a similar result. Multiple drug resistance (MDR) was observed in 264 (80.00%) of the 360 bacterial isolates. Only age was significantly associated with a culture-positive UTI. Conclusion: A higher prevalence of culture-positive UTI was detected. Escherichia coli was the most prevalent uropathogen followed by Enterococcus faecalis and Enterococcus faecium. These uropathogens showed highly resistant to the commonly used antibiotics. Moreover, MDR was commonly observed. Thus, empiric therapy is unsatisfactory as drug sensitivity always varies over time.