Pseudoxanthoma elasticum (PXE) is a rare, inherited connective tissue disorder with numerous systemic manifestations that include premature coronary artery disease. Coronary artery bypass grafting (CABG) is known to be beneficial in patients with PXE-related coronary artery disease. In these patients, however, the suitability of arterial conduits, including the internal mammary artery, has been controversial. We present a patient with PXE-related coronary artery disease who has had long-term patency of a left internal mammary artery (LIMA) graft after an off-pump CABG procedure in which LIMA and bilateral radial artery conduits were used.