Introduction:
Harlequin ichthyosis (HI) is the most dangerous form of the autosomal recessive congenital ichthyosis characterized with thickening of keratin part of the baby’s skin and a gross thick scaly appearance, which is triangular or diamond pattern.1 The name takes origin from its characteristic facial appearance as the face is pulled wide open in manner of a clowns smile. Marked ectropion and eclabium with absent or poorly developed ears and nose, mobility limitation of joints are some of the clinical features of HI.1 As the skin barrier is severally compromised there is excessive water loss and electrolyte abnormalities followed by temperature dysregulation and increase risk of infections. Because of these reason HI is usually fatal albeit aggressive management.