Netherton syndrome (NS) is a rare form of skin disorder characterized by extensive skin desquamation, hair shaft abnormality and atopic manifestations. We report a case of a two-year-old girl brought to our dermatology clinic by her mother, who had a generalized scaly skin lesion that started at birth. Her family history revealed a similar case in two of her sisters. A skin examination revealed diffuse serpiginous erythematous pruritic plaques, surrounded by double-edged scales beside her hair shaft defects. The patient was diagnosed with NS, and we began treatment using topical emollients, antibiotic and corticosteroid.