The authors relate the recent advances on the diagnosis of gargoylism emphasizing the value of biochemic and hematologic tests. Four cases are presented. The first one was considered as gargoylism plus hypothyroidism before employment of the recent methods of diagnosis; afterwards however it showed to be hypothyreoidism only. In the second case it was impossible to distinguish whether it was gargoylism or Nieman-Pick disease, as the Dorfman-Steiness test was not then available. The other two were actually gargoylism confirmed by laboratory tests. The authors enhance the importance of the citochemical blood analysis and the Dorfman-Steiness test.