BACKGROUND:Gallbladder cancer (GBC) is a common malignancy in our country; however, very limited data exist on this malignancy in Pakistan. METHODS:This is a retrospective analysis of all the admitted patients diagnosed with GBC or cholangiocarcinoma in between 1st January 1995 to 31st December 2007. RESULTS:A total of 245 patients were admitted with diagnosis of GBC or cholangiocarcinoma. Sixty seven percent were females. Right hypochondrial pain (70.6%) and jaundice (49.8%) were the commonest symptoms, followed by nausea and vomiting (11.8%), weight loss (13%), fever (18.8%), anorexia (9.8%) and ascites (3.3%). Gallstones were seen in 132 (53.9%) patients. Pathological diagnosis was confirmed in 155 (63.2%) patients, adenocarcinoma (94.8%) being the predominant type. Metastasis was seen in 204 (83.3%) patients, with liver and abdominal lymph nodes being the frequent sites of metastasis. Most of the patients presented to the surgeons (42.9%) and gastroenterologists (35.9%) at their first visit. Only 89 (26.3%) patients were referred to medical oncologists and 42 (16.7%) of the patients actually received chemotherapy. The patients who received chemotherapy cisplatin and gemcitabine demonstrated partial responses (40%). Common bile duct stricture was seen in 78 patients and stenting was successful in 73 patients. Fourteen (5.7%) patients are alive to date, one is receiving chemotherapy, and another is alive with advanced disease while 10 patients had incidental diagnosis after surgery. Of all 53.9% of patients have died and 38% are lost to follow up. CONCLUSION:Most of the patients with biliary cancers present late with advanced disease at our referral tertiary care hospital. Minority of the patients received chemotherapy and most of responses were observed with cisplatin and gemcitabine combination or capecitbine based therapy.