Background/Purpose: Reports on the late results of choledochal cyst excision w Methods: Of the 84 patients who had choledochal cyst who came under our care, 79 have had definitive surgery, three are awaiting surgery, one is being observed with Caroli's disease, and the parents of one child have refused surgery. Thirty-eight patients treated decades ago had internal drainage procedures. Since 1972, 41 patients have had cyst excision with hepaticojejunostomy using a 40-cm Roux loop without an antireflux procedure. Early complications in those who underwent cyst excision with hepaticojejunostomy included anastomotic leak in three patients who required reoperation, cholangitis in two, and fluid collection in the gall-bladder bed that required no intervention in one. Results: During a follow-up period ranging from 4 months to 17 years (mean, 8.5 years), anastomotic structure, cholangitis, and intrahepatic stone formation developed in two children after being well for 8 years and over 11 years. These children required additional surgical procedures to overcome their problems. Asymptomatic intrahepatic stones 2 years after cyst excision with hepaticojejunostomy developed in a third child. There was no mortality in the entire group that underwent cyst excision and they are all enjoying a good quality of life. Conclusions: Careful, long-term follow-up is important in children who have choledochal cyst excision with hepaticojejunostomy. Copyright (C) 1997 by W.B. Saunders Company.