We report a case of a patient with an elastic pseudoxanthoma (PXE) who presented an acute ischaemia at the left lower limb. The cause of such ischaemia was a thrombosis into the iliac and femoropopliteal arteries. Patient underwent a surgical procedure. The arteriopathy associated with a PXE rarely cause an arterial major occlusion. We did not found a case of acute arterial thrombotic ischaemia and PXE, treated with direct arterial revascularization in the reviewed literature.