Background: Congenital anomalies of kidney and urinary tract (CAKUT) are characterized by 
structural and functional abnormalities of kidney, collecting system, bladder and urethra. CAKUT 
identified in 20% to 50% of all fetal congenital anomalies and represent the cause of 40–50% of 
pediatric end-stage renal disease worldwide. Aim of the study: This study was performed to report 
the patterns and clinical presentation of infants and children with CAKUT. Methods: Patients with 
diagnosed with CAKUT, presenting to Minia University Hospitals over one year duration were 
investigated and categorized based on underlying renal structural/functional malformation and 
associated extra-renal anomalies. Results: this study revealed that the most common type of CAKUT 
detected was uretro-pelvic junction obstruction (UPJO). The commonest pattern of presentation of 
CAKUT was fever, and urological manifestation was only in quarter of cases, genital anomalies were 
the most common associated malformation and the most common complication was recurrent urinary 
tract infection. Conclusions: CAKUT is a clinically heterogeneous group of diseases with diverse 
clinical phenotypes. More efforts should be aimed at improving antenatal detection as well as 
classification with comprehensive reference to the clinical features of the diseases.