Abstract: Metaphyseal dysplasia (Pyle's disease) is rare autosomal recessive disease. Only few cases were reported around the world since 1930 when Edwin Pyle, an orthopedic surgeon from United States, first describes the disease. Pyle's disease is asymptomatic; it may be diagnosed incidentally during routine radiological examination for a genu valgus deformity. From orthopedic point of view, Pyle's disease requires no treatment except for deformity correction or management of fractures. Orthodontic intervention may be required for malocclusion and abnormal dentation.