A 30-year-old man with disseminated porokeratosis of Mibelli (DPKM) was treated with oral etretinate. The dose ranged between 75 mg/day and 50 mg/day for 21 weeks. An improvement of the lesions was observed, especially of very painful verrucous plaques of the left shin. No serious side effect was seen. The patient, who had been incapacitated, is now able to work. The benefit of long-term therapy of etretinate should be considered against its side effects.