Biliary stones may occur after liver transplantation, mostly in relation with biliary strictures. We describe an unusual case of biliary stone developing 18 years after liver transplantation and biliary-enteric anastomosis for biliary atresia, responsible for an acute cholangitis caused by obstruction of the jejunal loop. Surgical exploration allowed the removal of the stone and did not reveal any enteric stricture. Both biliary and enteric anastomosis were patent. An hepatobiliary scintigraphy performed 2 weeks after surgery demonstrated a delayed bile excretion through the jejunal limb and a focal accumulation of the tracer at the level where the stone was found. In conclusion, biliary stone formation could develop after liver transplantation and biliary-enteric anastomosis, in the absence of mechanical stricture, secondarily to bile stasis caused by functional disorder of the efferent jejunal loop. Hepatobiliary scintigraphy plays a central role in the diagnosis and comprehension of such a disorder.