The supernumerary kidney, also known as the accessory kidney, is one of the rarest congenital anomalies of the genitourinary system without sufficient epidemiological studies. It is defined as having more than two kidneys with each kidney having its own vasculature, collecting system, and capsule. To date, there are few cases documented in literature worldwide. We hereby present the first reported supernumerary kidney case in a preterm 2-month-old infant with dysmorphic features associated with an ectopic kidney and coarctation of the aorta. The coexistence of cardiovascular anomalies and genitourinary anomalies indicates a shared embryologic origin and genetic linkage, underscoring the importance of screening both systems when anomalies are identified in either. Newborn screening ultrasonographic examinations are crucial for detecting congenital anomalies, especially in infants with pertinent medical history or physical features, leading to better outcomes through timely diagnosis and intervention.
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