[The harlequin baby. Apropos of a case]

Harlequin foetus is a rare clinical entity and consists in the most severe form of congenital ichthyosis. Here below, we report on a new case of Harlequin baby born from consanguineous first cousin parents. The underlying molecular basis of harlequin ichthyosis is still not definitely determined. Surviving of few cases is due essentially to the use of a synthetic Vitamin A acid. The benefit of that treatment is still questionable.