Familial hypercholestremia is an autosomal dominant genetic disorder due to mutations in LDL receptor gene resulting in high serum cholesterol & accumulation in tissues. Familial hypercholestremia is an important risk factor for atherosclerosis & premature coronary artery disease. we report a case of 13 year old boy with history of multiple xanthomas and severe hypercholestremia. Both parents had hypercholestremia with family history of similar complaints & death of sibling. Lipid profile & Doppler study of carotid arteries showed abnormality. The child was treated accordingly (lipid converting agents & life style modification and was on followup.