Bile duct stones (BDS) are often suspected on history and clinical examination alone but symptoms may be variable ranging from asymptomatic to complications such as biliary colic, pancreatitis, jaundice or cholangitis. The majority of BDS can be diagnosed by transabdominal ultrasound, computed tomography, endoscopic ultrasound or magnetic resonance cholangiography prior to endoscopic or laparoscopic removal. Approximately 90% of BDS can be removed following endoscopic retrograde cholangiography (ERC)+sphincterotomy. Most of the remaining stones can be removed using mechanical lithotripsy. Patients with uncorrected coagulopathies may be treated with ERC+pneumatic dilatation of the sphincter of Oddi. Shockwave lithotripsy (intraductal and extracorporeal) and laser lithotripsy have also been used to fragment large bile duct stones prior to endoscopic removal. The role of medical therapy in treatment of BDS is currently uncertain. This review focuses on the clinical presentation, investigation and current management of BDS.