Introduction: Collodion babies are a rare entity with a myriad of outcomes and prognosis, relating to disorders of cornification. Congenital autosomal recessive ichthyosis (CARI) usually presents at birth, often as a collodion baby . This is a case of a possible CARI with severe ichthyosis. Case presentation: A baby girl, born preterm following spontaneous onset of labour at 32 weeks to healthy first time parents was noted to have a tight, shiny parchment membrane at birth. Facial distortion was present with bilateral eyelid ectropion, eclabium and fish-mouth appearance. The only risk factor was the consanguineous marriage between parents. On day 3 the membrane started peeling, revealing thick skin with rapid shedding and growth of skin, causing hypernatremic dehydration, and later hypoalbuminemia. Cannulation remained to be difficult due to the severe ichthyosis which progressed over the following days and weeks. The baby was managed with dermatology and ophthalmology follow up, emollients and lowpotency steroid application and eye care. There was good weight gain, but the ichthyosis was severe. At 2 months of age, a trial of oral retinoids was started with the parents' consent. However, there was no significant improvement, and the baby succumbed to an event of severe skin sepsis at the age of 4 months. Conculsion: The severity and prognosis of congenital forms of ichthyosis are dependent on the time of diagnosis and the type of ichthyosis. This was a severe case with poor prognosis despite early detection and multidisciplinary management.