A xanthoma is a localized collection of tissue histiocytes containing lipid and is usually associated with hyperlipidemia. Tendinous and tuberous xanthomatosis have been found in association with familial hypercholesterolemia, Type III hyperlipidemia, beta-sitosterolemia and cerebrotendinous xanthomatosis. Tendinous and tuberous xanthomatosis without hyperlipidemia have been reported very rarely. Especially tendinous xanthomatosis without hyperlipidemia have not been reported at all in korea. We are reporting a patient with tendinous and tuberous xanthomatosis accompanied by normal plasma lipids.