Summary The efficacy of tazarotene 0.1% gel in a 30-year-old woman with type I lamellar ichthyosis is reported. The drug was applied to 15% of the total body surface area as follows: once daily for 2 weeks, three times a week for further 2 weeks, followed by a once weekly maintenance application. During the first week of treatment there was partial improvement obtained and in the next 14 days further reduction of scaling within the tazarotene-treated areas was observed. After 4 months of maintenance application, there was a marked overall improvement in the treated areas. Side-effects consisted only of mild pruritus, slight burning and irritation. In essence, the therapeutic benefit obtained was comparable with that of systemic retinoids but without the adverse systemic side-effects. As noted in other reports, tazarotene 0.1% gel seems to be a valuable and safe therapeutic option for this severe genodermatosis.