SUMMARYEngelmann's disease or progressive diaphyseal dysplasia is a rare disease. We report a Malaysian Chinese girl with typical radiological manifestations characterized by gross thickening and sclerosis of the skull and long bones. In her case, pulmonary tuberculosis was also present. Various laboratory investigations showed a low haemoglobin and a raised erythrocyte sedimentation rate. Other results were normal. Bone marrow biopsy from the iliac crest showed a sideroblastic type of haemopoiesis and this was thought to be abnormal, for it was felt that the drug isoniazid was too recently administered to cause any bone marrow changes. Pathogenesis of this disease still remains unknown.
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