Background:Ichthyosis linearis circumflexa is a rare form of ichthyosis characterized by polycyclic and annular lesions which are bordered by a double-edged scale. Netherton syndrome is a genodermatosis in which ichthyosis linearis circumflexa is accompanied by characteristic hair shaft abnormalities and atopic diathesis. Different treatment modalities such as emmolients, keratolytics, calcipotriene, topical corticosteroids, topical calcineurin inhibitors, systemic retinoids, phototherapy and photochemotherapy have been used with variable results. Topical therapies may result in considerable absorption because of the defective barrier function in ichthyosis linearis circumflexa/Netherton syndrome.Main observation A 12-year-old patient diagnosed as ichthyosis linearis circumflexa demonstrated considerable improvement with narrowband UVB phototherapy at the end of 30 sessions.Conclusion:Short-term narrowband-UVB may an effective treatment option in patients with ichthyosis linearis circumflexa/Netherton syndrome.