Celiac stenosis or occlusion is attributed partly to increase blood flow at pancreatic arcade from the superior mesenteric artery (SMA) system and may play a causal role in true aneurysm of pancreaticoduodenal artery (PDAA) formation. However, despite possible increased blood flow in the pancreatic arcades like celiac stenosis, PDAAs with a stenotic SMA are extremely rare, with only three cases have been reported in the literature. We report a case of PDAA with SMA stenosis and review the literature.