A 45-year-old female patient was admitted to the emergency department with syncope. Her medical history revealed a diagnosis of Familial Partial Lipodystrophy 2 (FPLD2). The patient's electrocardiogram showed a complete atrioventricular (A-V) block, and she had a history of insulin-dependent diabetes mellitus and coronary artery bypass surgery. A severe stenosis was observed in the aortic right coronary artery saphenous vein graft during coronary angiography, which was successfully revascularized. Subsequently, due to persistant syncope attacks, a permanent pacemaker was implanted after an electrophysiological study. This case highlights that serious cardiac conduction defects in patients with FPLD2 may not only be related to coronary artery disease but can also present as direct conduction defects.