Genistein, a soy isoflavone, reduces glycosaminoglycan synthesis and its effect on mucopolysaccharidoses has been tested. In this report, we examined the effect of genistein in human mucolipidosis II skin fibroblasts in vitro. Heparan sulfate was accumulated within both cells and in extracellular spaces in mucolipidosis II. Genistein reduced the amount of heparan sulfate in cultured cells dose dependently and also inhibited cell growth dose dependently.