Background: Lamellar Ichthyosis (LI) is a rare hereditary autosomal recessive disease with a defect in chromosome 14q11 which generates defect of transglutaminase-1 (TGM-1) enzyme. Clinical manifestation of IL is initiated with the occurrence of collodion membrane that sheds and is subsequenly replaced by thick scale, eclabium, ectropion, and palmoplantar hyperkeratosis. Gold standard of LI diagnosis is genetic analysis examination. Ichthyosis therapy includes scales removal, topical keratolytic, oral retinoid, and its complication management.