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Netherton syndrome associated to A 7-year-old girl born to consanguineous parents, had recurrent erythroderma since birth; she presented with intractable pruritus, scaling, dry skin and eczematous lesions, associated to fingers and toes onychogryphosis, along with refractory otitis. The hair was sparse and brittle, the simple light microscopic examination of hair shaft revealed a pathognomonic Bamboo aspect (trichorrehxis invaginata) and ear swab culture revealed Candida parapsilosis as otitis agent. Due to the lack of genetic routine testing, children with recalcitrant erythroderma and otitis, along with hair shaft abnormalities, need to be evaluated for Netherton syndrome.