Harlequin Ichthyosis is the most severe form of congenital Ichthyosis presenting at birth. It is characterized by thick, fissured armor-plate hyperkeratosis, ears and nose deformities, ectropion, eclabium with fish mouth appearance, flexion deformities of all joints and hypoplastic digits. It is a very rare disorder with autosomal recessive inheritance. Perinatal mortality is high and the survivors develop severe erythroderma subsequently. We report a case of Harlequin Ichthyosis not only because of its rarity but also its tendency to occur in consecutive pregnancies. We recommend to have a genetic screening and counseling in all high risk couples e.g. consanguinity marriages as well as having more studies to diagnose and determine the best mode to deliver a baby with HI. Conclusion: Early diagnosis and genetic counseling of the parents is an important step in managing Harlequin Ichthyosis. We recommend to have a genetic screening and counseling in all high risk couples e.g. consanguinity marriages, and with family history of Harlequin icthyosis or previous child born with Harlequin icthyosis preimplantation genetic diagnosis can be done by screening for ABCA12 gene in both affected baby and parents.