Harlequin fetus is the most severe form of congenital ichthyosis with an incidence of one in 300,000 births.An autosomal recessive pattern of inheritance is seen in this disorder.However,a new autosomal dominant mutation may possibly be responsible.The disorder has an ominous prognosis since the neonates usually die in the first hours or days of life.We have reported here a case of harlequin fetus with a history of Psoriasis in his father .