Buschke-Ollendorff syndrome is an association of connective tissue nevi and osteopoikilosis that usually appears in the first decades of life. The nevi occur on the trunk and extremities. In a 48-year-old man with this rare syndrome the connective tissue nevus was located on the scalp and was pruritic. Histologically, involvement of the epidermis with cystic dilatation of the hair follicles and an abnormality of mucopolysaccharides were present. The nevi in this syndrome may involve an alteration in the three components of connective tissue to a variable degree.