Confluent and reticulated papillomatosis is a fairly rare dermatosis of still unknown origin affecting mostly female young adults. The lesions are mainly localized in the midline of the trunk. Systemic treatment is the treatment of choice because the disease is resistant to topical therapy and recurrences are often seen. In recent publications retinoids and minocycline have been reported as the favourite therapeutic approaches. We successfully treated a 19-year-old-girl with azithromycin resulting in a complete healing of all skin lesions. The patient has been free of disease for five months. Based on our own case and data in literature, azithromycin is an effective, reasonable and safe therapeutic alternative.