Cystic biliary atresia (CBA) is a relatively uncommon cystic variant of type 1 Kasi classification of biliary atresia. It usually presented with cholestatic jaundice, elevated liver function tests, and ultrasound finding of a cyst at the hepatic hilum, which mimics the features of a choledochal cyst (CC). We present the first reported case of CBA in Saudi Arabia with the imaging findings and surgical outcome. A 53-day-old infant admitted to the neonatal intensive care unit (NICU) due to persistent jaundice since birth. Imaging findings and surgical exploration confirm the diagnosis of CBA. An excellent prognosis of CBA with early surgical intervention necessitates the radiologist to be familiar with this entity and differentiate it from CC.