Hypertrophic osteoarthropathy is a rare syndrome characterized by dysacromelia and periostosis with digital clubbing. Primary form is called pachydermoperiostosis. A case of a primary hypertrophic osteoarthropathy without pachydermia is reported in a 13-year-old boy, which was born of a consanguineous marriage. This case suggested an incomplete form of pachydermoperiostosis with a recessive inheritance. Pachydermoperiostosis is often familial and occurs predominantly in men. This affection is believed to be inherited in an autosomal dominant pattern but some autosomal recessive forms have also been reported.