Introduction. Congenital anomalies of the ureters in children are a significant concern in pediatric urology. One such anomaly is the double kidney, where there are two non-delimited or partially delimited parenchymal masses, each with their own collecting system and ureter. This condition results from the division of the metanephrogenic blastema of the ureter during development. The incidence rate of this anomaly is 2:1 in females, and it can be asymptomatic or present with various symptoms, such as fever, hydronephrosis, colic, and renal lithiasis. Diagnosis is established through prenatal or postnatal ultrasound, urography, and cystoscopy. Aim of study. To present the clinical case of a patient with a double bilateral kidney, bilateral megaureter, ectopic ureterocele, and nonfunctional upper renal pelvis on the right. Material and methods. Analysis of the specialized literature in terms of the clinical case of a patient with renourinary congenital anomaly, through the SIAMS intra-hospital search engine. Results. The patient, a 2-year-old, presented with abdominal pain, leukocyturia, urinary hesitancy, fever, pallor, and loss of appetite. Ultrasound of the urinary system revealed bilateral double kidneys, hydronephrosis on the right, and ectopic ureterocele. The patient underwent surgical intervention, including heminefroureterectomy and ureterocelectomy of the upper renal pelvis on the right. Conclusions. The correction of congenital reno-urinary malformations in children remains an important issue in pediatric urology. However, advances in technology and treatment have improved outcomes for children with these conditions.