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Lamellar Ichthyosis is a rare genodermatotic condition, which occurs due to the mutation in the transglutaminase-1gene. It is a rare disease with a global prevalence of 1 in 300000 live births with no specific gender predilection. It is a rare genetic disorder with a characteristic fish scale appearance of the skin. Here we report one such rare case of Lamellar Ichthyosis in a 7-year-old girl reported to us with difficulty in chewing.