The authors critically review the literature in order to ascertain the current state of knowledge regarding the anatomopathological, clinical and therapeutic characteristics of choledochocele, or cystic dilation of the terminal portion of the biliary tract. In particular, the Authors examine the etiopathogenetic aspects to explain how the presence of a choledochocele may lead to the onset of chronic pancreatitis. A clinical case which was recently brought to their attention is examined in detail. It concerns a 27-year-old patient in whom the presence of a large choledochocele had led to the onset of severe cephalopancreatitis which resisted all forms of treatment. In the case in question, ERCP played a decisive role in the diagnosis of the choledochocele, whereas ultrasonography, CT and selective arteriography were useful above all in relation to pancreatitis. In the case described ultraradical surgery, namely duodenocephalopancreatectomy, allowed the severe pancreatopathy and its etiological cause (choledochocele) to be efficaciously treated, confirming that this type of surgery currently plays an important role in the treatment of chronic pancreatitis.