Harlequin ichthyosis is a rare and exceedingly severe form of congenital ichthyosis with an incidence of approximately 1 in 300,000 births. These patients are at a high risk for neonatal
infection and septicemia. Most affected infants die within the first days or weeks of life.
We report a male baby born with harlequin ichthyosis. There is limited information regarding the course and prognosis of neonates affected with harlequin ichthyosis. However, it is now evident that these infants, depending on the severity, may have an extended survival potential with intensive supportive measures as well as the addition of retinoids.