Eighty-four patients with choledochocele collected from the world literature and one personal observation are reviewed. The main issues regarding clinical presentation, diagnostic work-up, and the treatment of this uncommon lesion are discussed. Abdominal pain was the most common clinical feature (91% of cases), followed by pancreatitis (38%), nausea or vomiting (35%), and jaundice (26%). In addition, associated lithiasis was found in 43% of the cases. Endoscopic retrograde cholangiopancreatography was the most useful diagnostic procedure and resulted in a correct diagnosis in all but one of the patients investigated by this method. Surgical excision of the duodenal luminal portion of the choledochocele was the treatment most commonly used (65% of cases). In recent years, operative endoscopy has also been increasingly used, with good results.