Byler disease associated with acute cholecystitis.

A case of Byler disease, which has a rare incidence of l in 90000, is reported. A 21-year-old woman known to have the disease since childhood presented with acute cholecystitis and underwent laparoscopic cholecystectomy. To the best of our knowledge, after a review of the English literature, this is the second patient with Byler disease to live beyond the second decade of life since the disorder was first described by Clayton 30 years ago, and the first case associated with acute cholecystitis that was managed successfully by laparoscopic cholecystectomy.