Scleroderma en coup de sabre, a variant of localized scleroderma is a disorder characterized by fibrosis of connective tissue. We report a 21-year-old female with scleroderma en coup de sabre accompanied by pachydermoperiostosis. She was born to consanguineous parents and her older sister also had pachydermoperiostosis characterized by clubbing of the digits, enlargement of distal parts of the extremities. The two disorders were diagnosed by clinical examination, histological and x-ray findings. In contrast to scleroderma, pachydermoperiostosis is a hypertrophic process characterized by periosteal proliferation of the tubuler bones and hypertrophic skin changes. We discuss this interesting coexistence and review the literature.