BackgroundA double major papilla of Vater is a rare congenital anomaly with only three documented cases described in the literature.Case reportWe report the case of a 19-year-old man, with chronic ulcerative pancolitis and congenital sphrerocytosis, who underwent endoscopic retrograde cholangiopancreatography because he had persistent elevation of liver enzymes and normal MRI cholangiography. During endoscopic retrograde cholangiopancreatography, a double papilla of Vater with separate drainage for the bile duct and the pancreatic duct was observed.ConclusionEndoscopic retrograde cholangiopancreatography showed normal pancreatogram and findings compatible with sclerosing cholangitis.