Introduction: Congenital pancreaticobiliary anomalies (PBA) are rare and may be classified into various subtypes. This study defines their incidence, associations with biliary and pancreatic diseases, and treatment options. Methods: Retrospective examination of records and films of 5081 patients (pts) who underwent endoscopic retrograde cholangiopancreatography (ERCP) by a single endoscopist in an urban medical center from 1972-2005. PBA was grouped as 1) pancreatic divisum (PD), 2) congenital biliary dilatation (CBD), 3) anomalous pancreaticobiliary ductal union (APBDU) and 4) other congenital pancreaticobiliary anomalies (OPBA). Results: Congenital PBA was seen in 209 pts including 157 pts with PD, 22 pts with CBD, 14 pts with APBDU and 16 pts with OPBA. 1. Associated findings in the 157 PD pts included pancreatitis (54 pts), biliary lithiasis (20 pts). There were associations with other pancreaticobiliary maljunction (2 pts), CBD (1 pt), gall bladder cancer (4 pts), ampullary cancer (3 pts) and pancreatic cancer (2 pts). Treatment included major sphincterotomy (5 pts), minor sphincterotomy (1 pt), and Whipple's procedure (2 pts). 2. The 22 CBD were subgrouped into columnar types (4 pts), cystic type (5 pts), and spindle type (13 pts). Treatment included cholecystectomy with cholangioduodenostomy (3 pts), sphincterotomy (3 pts), cholecystectomy alone (1 pt) and choledochoectomy with hepatocholangiojejunostomy (1 pt). 3. There were 14 pts with APBDU. 5 pts had associated dilation, whereas, 9 pts had normal sized duct. Associated conditions included cholelithiasis (2 pts), sickle cell disease (2 pts), pancreatitis (3 pts), choledocholithiasis (1 pt) and gallbladder cancer (1 pt). Two pts also had PD. Treatment included sphincterotomy (2 pts), cholecystectomy (2 pts), cholecystectomy and lymphadenectomy (1 pt), and choledochectomy with hepatocholangiojejunostomy (1 pt). 4. The 16 OPBA were choledochocoele (8 pts), Caroli’s disease (4 pts), double main pancreatic duct (2 pts), double gallbladder (1 pt), and cystic duct diverticulum (1 pt). Conclusion: Congenital pancreaticobiliary anomalies are rare and often overlooked. PD, the most common congenital PBA, is often associated with pancreatitis, cholelithiasis, and choledocholithiasis. There is also a correlation with pancreaticobiliary cancer, APBDU, and CBD. Both CBD and APBDU are associated with PD and with gallbladder cancer. Surgical treatment is indicated for cancer and symptomatic pts. Appreciation of PBA is important for proper diagnosis and treatment strategies.