Pachydermoperiostosis or primary hypertropic osteoarthropathy is a very rare genetic disease. This syndrome is characterized by gyrus formation on face and scalp, hypertrophy of soft tissue, clubbing of digit, seborrhea, and thickening of periosteum.  This disease is clinically similar to secondary forms of hypertropic osteoarthropathy, which is usually seen in pulmonary disease or cyanotic congenital heart disease. The facial gyri which gives bad impression, the patient becomes socially isolated. To correct this problem, authours introduced combined theraphy(excision, demabrasion, isotretinoin) for pachydermoperiostosis and satisfactory results could be obtained aesthetically and psycologically without any complications.