Two cases of a rare form of congenital ichthyosis in infants born to the same parents are presented. The report is made possible by the fact that the same family has been coming to the same hospital for years. In this family it was evident that the skin defect was sexlinked. On March 27, 1954, a white, 20-year-old woman in Barcelona, Venezuela, gave birth to a boy, with all the characteristics of a severe congenital ichthyosis. The boy was a full-term male, well developed, who cried at birth, and seemed to be active. Pregnancy and delivery were completely normal. This child had a very thick, yellow hard skin, with red deep crevices going through all thicknesses of the skin and presenting a striking contrast (Fig. 1). It looked like the fetus described as "the harlequin." There was no bleeding. Ectropion of the conjunctiva was present. The fingers and toes were