Harlequin ichthyosis, or harlequin fetus, is a relatively rare, severe form of congenital ichthyosis. Although the clinical features have been well described, very little is known about the pat ho genesis, clinical course, appropriate treatment and prognosis of harlequin ichthyosis because most of these babies with harlequin ichthyosis die within a few weeks of life. We herein report a case of harlequin ichthyosis, which we believe is the first such case in Taiwan.
