[Buschke-Ollendorff syndrome. Connective tissue nevi in osteopoikilosis]

We describe here a patient with Buschke-Ollendorff syndrome (BOS) and present a review of the literature. BOS is characterised by osteopoikilosis, small sclerotic changes in the bones, and by dermatofibrosis lenticularis disseminata, yellow-white nodules in the skin. It is a benign, hereditary condition, with no influence on the patient's health. Awareness of this condition is of importance, as the wrong diagnosis can lead to unnecessary worry for the patient and extensive costs for the health care system.