Median arcuate ligament syndrome (MALS) involves narrowing of the celiac artery root from MAL compression, leading to pancreatoduodenal artery aneurysm (PDAA) due to increased retrograde blood flow from the superior mesenteric artery into the PDA. We encountered a case in which coil embolization was performed for PDAA rupture due to MALS. Four years later, a second PDAA occurred and ruptured, necessitating coil reembolization. There have been no reports of recurrence during long-term follow-up after PDAA treatment in patients with MALS. We herein report a rare case of metachronous PDAA rupture in the context of MALS. The relevant literature and 11 PDAA/MALS cases are discussed.