Birt-Hogg-Dubé-syndrome, first described 20 years ago, is an autosomal dominant inherited disease, characterized by the occurrence of multiple cutaneous hamartomas with adnexal differentiation. An association with intestinal neoplasms has been mentioned repeatedly. Recently, the cutaneous lesions in this syndrome have been interpreted as different developmental stages of one single hamartoma with sebaceous differentiation, called mantleoma. Numerous dermatohistopathologic studies contribute to the certain diagnosis of Birt-Hogg-Dubé-syndrome. In contrast, little information is available on the therapy of the multiple skin lesions. A father and his daughter with Birt-Hogg-Dubé-syndrome were treated with the CO2 laser, producing satisfactory results.