We present a rare case of Lamellar Ichthyosis with bilateral ectropion with left sided corneal ulcer with descemetocele in a four-month-old female child, the youngest ever reported. Ichthyosis is a group of skin disorders characterized by the presence of fish-like scales all over the body (Mushriff, 2016; Turgut et al., 2009). Lamellar Ichthyosis is a rare congenital condition with incidence of 1 in 100,000 (Conditions), affecting males and females equally. Inheritance is Autosomal Recessive (Conditions). It involves generalized body as a collodion baby at birth; once the membrane sheds, patient develops large, thick, brown, pasted scales associated with ectropion, eclabium, scarring alopecia, plantar and palmar hyperkeratosis (Mushriff, 2016). Mild cases are managed with hydration, lubrication and keratolytic-agents (Mushriff, 2016). Severe cases are treated with Oral Retinoid. Proper lubrication and patching of eyes with timely management of ectropion to prevent the exposure keraopathy and related complications are needed in such cases. Secondary infections can lead to vision threatening complications, so child with ichthyosis should be under Ophthalmic observation for early needed interventions.