Introduction: Gallbladder agenesis (GA) is a rare congenital anomaly, with a reported incidence ranging between 13–65 % per a 100 000 population. Its association with acute pancreatitis is extremely rare. Objective: To present a case of GA associated with recurrent acute pancreatitis. Case Presentation: We present an 85 years old woman whose clinical record reported a bladder stonediagnosis by US, that arrive to the hospital with frequent episodes of intermittent vomiting, abdominal pain and hypotension. She was admitted at hospital with upper abdominal pain, vomiting and hypotension. At the physical examination the abdomen was soft, with mild epigastric tenderness, without tenderness rebound or tumor. US report gallbladder not visible. In less than 24 hours the patient evolved with shock, multiple organ failure and death. Gallbladder agenesis and acute pancreatitis were found in autopsy. Conclusions: The GA is a rare congenital anomaly, difficult to establish a correct preoperative diagnosis, always found during the abdominal surgery or autopsy. Its association with acute pancreatitis is very uncommon. Pancreatitis may be due to of Oddi Sphincter’s Dysfunction (SOD) or idiopathic origin. Keywords: Gallbladder agenesis, biliary anomaly, acute pancreatitis, of Oddi Sphincter’s Dysfunction, idiopathic origin.