The authors present the observation of an adult man followed up on a long-term basis, suffering from Camurati-Engelmann progressive diaphyseal sclerosis with a number of typical osseous manifestations (sclerosis of the diaphyses, hyperostosis, deformity of the joints, poor posture) and clinical symptoms (muscular weakness and conversely contractures, deafness, Méniere’s disease). A less frequently encountered finding in the patient is massive affection of the skeleton of the head and the picture of metaphyseal dysplasia on the lower extremities of the femurs. Both these phenomena indicate possible associations of different syndromes of sclerotizing osseous dysplasias. The valgose deformities of the legs are gradually eliminated by corrective osteotomies, which heal well.

        Key words: Camurati-Engelmann disease - progressive diaphyseal dysplasia - intrinsic osteoscleroses