The effect of the aromatic retinoid, etretinate, has been evaluated in 17 patients suffering from moderate to severe Darier's disease (DD). The erythema, hyperkeratosis and number of papules as well as the extension of the lesions were scored before and at regular intervals during treatment. In the first treatment period a daily dose of 50 mg was given until healing but no longer than 10 weeks. In the majority of patients the effect was very good with almost complete clearing in 6 patients. In patients with severe DD a reduction of the initial score by more than 50% was seen in 7 out of 8 patients. Isomorphic reactions developed in 6 of 17 patients and was the most important side effect. In patients with moderate disease the remission periods without treatment exceeded 4 weeks. 8 patients were treated intermittently with a lower dose up to 4 treatment periods. Equally good results were obtained. The treatment of choice in DD seems to be etretinate at an intermittent regime with low doses (less than or equal to 0.5 mg/kg).