In order to review current knowledge of BOS and its cutaneous manifestations, a PubMed survey using the search terms “BOS”, “Ollendorf Buschke”, “Buschke-Ollendorf”, “osteopoikilosis”, “melorheostosis”, “LEMD3”, and “Man1” was undertaken (Table 1). This evidence concerns the gene LEMD3 and Buschke-Ollendorff syndrome.