Ichthyosis refers to a relatively uncommon group of skin disorders characterized by the presence of excessive amounts of dry surface scales. It can be congenital or acquired. Our patient was third child with this disorder and out of three none survived. In congenital ichthyosis form of disorders, routine histopathology, electron microscopy, and frozen sections of skin biopsy specimens may be required to determine the specific classification of disease. The mainstay of therapy includes removal of surface scales and application of a water barrier. In disabling cases, oral retinoids may reduce cosmetic disfigurement, depression, and social isolation.