Introduction: Netherton Syndrome (NS) is a rare hereditary autosomal recessive disorder with ichthyosiform cutaneous lesions, specific hair shaft defect and atopic diathesis. The incidence of Netherton syndrome is estimated to be approximately 1 in 200,000. The objective of this case report is to illustrate the Netherton Syndrome in a patient with severe eczema atopic dermatitislike eruption. Case presentation: A 41-month old boy referred to the clinic of Allergy and Immunology, Hazrat Rasoul Hospital with generalized erythema and scaling cutaneous lesions. The patient underwent clinical examinations and laboratory analysis. Laboratory data revealed only an elevated IgE level, leukocyte count was 7800/μl with 10% eosinophil, and his hair shaft indicated classic trichorrhexis invaginata (Bamboo hair). Based on the clinical and laboratory findings, N.S was diagnosed. A brief review was also done related to this case. Conclusion: The case was reported because of the severity of the disorder and other differential diagnoses in severe and refractory atopic dermatitis-like eruptions. The importance of the case is related to other differential diagnoses, especially with early onset disorders at neonatal age.