Spontaneous perforation of extrahepatic bile duct is a rare entity, but is the second cause of surgical jaundice in the newborn period after biliary atresia. We review the etiological theories and three patients with this anomaly. Centrolobulillar cholestasis was present in all cases. Parietal ischemic necrosis was seen in one case, obstructive lesions (extrahepatic biliary atresia) in two and malformative lesions (choledochal cyst) in the last patient. A developmental error and a small circumscribed area of duct wall weakness have been the most accepted factors involved in its pathogenesis. However we have found histologic and morphologic evidence that ratify the obstructive and vascular lesions as relevant elements in the etiology of the spontaneous perforation of the biliary tract.