Ichthyosis which is an inherited keratinisation disorder of the skin, is heterogeneous as clinically. This disease’s clinic is considerably variable from widespread skin dryness to hyperkeratosis. While the prevention of complications such as dehydration, sepsis and electrolyte imbalance, is very important in respect to survival, in premature neonates, more severe complications may be seen. In this case, we reported that a premature infant who developed catastrophic candida sepsis with congenital ichthyosis.