Background: Congenital abnormalities of the kidneys and urinary tracts (CAKUT) are conditions brought on by errors in the growth of the kidneys and the outflow tubes that drain the kidneys. Although the issue may not become apparent until later in life, CAKUT is a congenital abnormality brought on by improper urinary system development. Case presentation: We are presenting the case of a 23-year-old man who has chronic kidney disease and is undergoing maintenance hemodialysis. The patient was apparently alright 2 weeks back when he had complained of breathlessness. fever 2 episodes, lower which was insidious in onset and gradually progressive. On arrival, the patient was provided oxygen therapy via a face mask for breathlessness and to make him feel comfortable with the head elevation. On laboratory investigation, the patient’s blood values are altered. The patient underwent computed tomography of the kidney-ureter-bladder (CT-KUB), and Ultrasonography of the kidneys (USG). A permanent tunnel hemodialysis catheter under local anesthesia was placed and he received 2 cycles of hemodialysis. During the course in the hospital, the patient’s condition was improving and was stable clinically and vitally hence, the patient was discharged. Conclusion: CAKUT can occur alone or in conjunction with heart and cardiovascular malformations, gastrointestinal tract malformations, central nervous system malformations, skeletal system malformations, lung, face, genito-reproductive system malformations, abdominal wall malformations, chromosomal abnormalities, MCA, and other organ system malformations.