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A unique case of an asymptomatic newborn with type I choledochal cyst and a single umbilical artery is reported. Ultrasound imaging was used as a screening tool and magnetic resonance cholangiopancreatography as a method of adequate visualization. On day 8 of life, the patient had a laparotomy with resection of choledochal cyst, cholecystectomy, and recon- struction using a jejunal Roux-en-Y to portal plate.

Type I choledochal cyst – an atypical presentation and an unusual association with single umbilical artery