Harlequin ichthyosis is a rare autosomal recessive congenital ichthyosis with a distinct phenotypic appearance. It associated with a high mortality rate and affects both sexes equally. We report a harlequin fetus with a history of scalp psoriasis in his mother. The neonate was born to consanguineous parents who had a previous female baby that was diagnosed with harlequin ichthyosis.

Harlequin ichthyosis is a rare autosomal recessive congenital ichthyosis with a distinct phenotypic appearance. We report a male baby with harlequin ichthyosis who was born to consanguineous parents that have a previous female baby who was diagnosed with harlequin ichthyosis.