RESUMO
Engelmann-Camurati's diseases is a progressive alteration of the diaphysis of the long bones, where the etiology is unknown. It appears clinically around the age of two years, showing weakness of the lower limbs and frequent falls; there is also weight loss and in older patients, the appearance of secondary sexual characteristics is retarded. The diagnosis is made clinically and corroborated with x-ray studies that show increased density and width of the diaphysis. We report a case in a female where the diagnosis was established at twelve years of age; she received steroid therapy for four years without improvement. The mechanism of action of the steroids is unknown, but it seems that they stimulate the formation of healthy bone with adequate remodelling. Physiotherapy is necessary in these patients and if there should be a compression of the cranial nerves, surgical management may be useful to relieve compression.