Introduction: Spontaneous extrahepatic bile duct perforation is rare in newborns.It is a surgical cause of jaundice in this period and the acute presentation is unusual.Objective: To report a case of spontaneous bile duct perforation in a newborn due to its serious complications if an early and timely diagnosis is not performed.Clinical case: A 10-day-old newborn who developed food rejection, fever and abdominal distension without jaundice, acolia, or coluria two days prior of admission.The laboratory tests showed leukopenia, thrombocytosis, increased C-reactive protein, and normal hepatic function.The abdominal x-ray showed pneumoperitoneum, and the diagnosis of necrotizing enterocolitis was made.Laparotomy was performed; extrahepatic bile duct perforation and biliary peritonitis were noted.Intraoperative cholangiography demonstrated rescatable proximal bile duct and dilated cystic duct.Hepatic-jejunostomy was performed with Roux-en-Y and cholecystectomy.In the postoperative study portal thrombosis was found, so he received anticoagulant treatment.At 8 months of age, the patient had enteral feeding tolerance and adequate weight gain.Conclusions: Biliary perforation is a rare entity and more in the neonatal period, a condition that makes it a diagnostic and therapeutic challenge.The prognosis will depend on early intervention and intraoperative findings.