Hyperlipoproteinemia may lead to early development of atherosclerosis especially when present in familial forms. Serum low-density lipoprotein (LDL) cholesterol is known to be the most important form of atherosclerotic particules.  Type IIa hyper-lipoproteinemia is one of the most important form of these familial disorders and gives rise to extremely elevated serum LDL cholesterol levels. Beside the cardiovascular mortality risk that the patient carries because of the early development of atherosclerotic lesions in all vascular tree, this clinical entity also manifests itself with accumulation of cholesterol particules on the skin. Herein we report a very early presentation of Type IIa hyperlipoproteinemia with both atherosclerotic lesions in the arteries and lipid deposites on the skin.