Buschke-Ollendorff syndrome is characterized by the coincidence of dermato-fibrosis lenticularis disseminata and focal sclerotic bone dysplasia (osteopoikilosis). The case of a 39-year-old female is presented and the characteristic clinical, histopathological and radiological manifestations of this rare disease are reviewed. As focal bone lesions in Buschke-Ollendorff's syndrome are mostly asymptomatic, the rate of diagnosis could be increased if locations of predilection were subjected to X-ray examinations on clinical observation of the typical cutaneous manifestations.