Abstract19 year male presented to us with plethora of tendinous and tuberous xanthomas over hands, elbows, knees, ankles, dorsum and sacral area since last eight years. Fasting lipid profile revealed very high total cholesterol(TC) and serum LDL consistent with Type IIa hyperlipoproteinemia. Screening of the parents and family members revealed no derangement of lipid profile in any of them. Although conventionally described as autosomal codominant, we report a rare case of de novo familial hypercholesterolemia which results in premature coronary death and timely intervention only prevents premature atherosclerosis.