Netherton’s syndrome is a rare, autosomal recessive disorder or keratinization characterized by trichorrhexis invaginata, inchthyosis linearis circumflexa and atopic diathesis. There is also failure to thrive, Recently the genetic defect has been identified as a mutatin in SPINK5 gene on chromosome 5q31-q32. A case of this rare disorder with classical presentation in a 16-year-old boy is reported here. He had congenital erythroderma, failure to thrive, ichthyosis linearis circumflexa, trichorrhexis invaginata, epilepsy, spastic gait, and raised IgE levels. A brief review of the literature is presented as well.