Agenesis of the gallbladder

Agenesis of the gallbladder, with normal bile ducts, is a rare congenital condition occurring in 13 to 65 people out of a population of 100,000 and probably results from a failure of the gallbladder bud to develop or vacuolize in utero. Reports of a familial tendency toward this condition may suggest screening of asymptomatic family members and speed treatment when symptoms manifest, thus avoiding surgery. Choledocholithiasis in patients with gallbladder agenesis is rare, the stones found are usually small, and patients had invariably first presented with jaundice. The authors describe a case of a 23-year-old patient with gallbladder agenesis and a large choledochal calculus and without jaundice at presentation. A review of the literature is presented.