Among the various gastrointestinal manifestations observed in patients with cystic fibrosis (CF), gallbladder abnormalities occur frequently. These include a high prevalence of nonfunctioning gallbladders (30%), micro-gallbladders (8-30%), and gallstones (4-30%). The underlying pathophysiology for this increased prevalence in patients with CF is not completely understood, due to contradictory findings. These findings concern: (1) abnormalities in bile acid metabolism resulting in bile that is supersaturated with cholesterol, (2) an impaired nucleation time, and (3) biliary stasis, due to bile duct abnormalities and/or impaired gallbladder motility. The diagnosis of gallbladder disease in CF may be obscured by other common gastrointestinal complications, resulting in a long delay between onset of symptoms and the diagnosis. Cholecystectomy in CF is the treatment of choice, provided they are carefully managed in the pre- and perioperative period. The operative morbidity and mortality, even with intensive management of pulmonary disease, amounted to 10% and 5%, respectively. Therefore, alternative options, like laparoscopic cholecystectomy are of interest and require further investigation, especially for CF patients with severe pulmonary disease.