Case report of a Hunter-Hurler syndrome (gargoylism). The diagnosis was based on the clinical picture, although the grotesque facies, one of the main characteristics, was missing. The presence of typical vertebral alterations and the milk diffused opacity of the cornea, are sufficient to assure the diagnosis. The electrophoretic analysis of the serum proteins do not allow the authors to make definite statements because only one case was studied; however they point out the fact, considered very important by them, that there was a lipoprotein without electrophoretic mobility which indicating the presence of an instable lipoprotein complex in the blood, may suggest the possibility of lipoid deposit formation in the cells.