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A 67-year-old woman was admitted because of a progressive impairment in her visual fields during the last six days, together with bilateral fronto-orbicular cephalea, particularly in the morning. In 1980 she had been diagnosed as suffering from diabetes insipidus. For this, she received chlorpropamide and clofibrate, and then inhaled desmopresine. A radiological study performed in 1986 because ofpain in the left knee confirmed the existence ofan osteolytic image at this level, together with lytic images in both iliac crests and the left scapula. Skin hyperpigmented lesions with thickening of the epidermis were detected in both legs, and the biopsy of one of these lesions also showed a typical histological pattern of histiocytosis. Treatment with etoposide and prednisolone was given, but had to be discontinued because of side-effects.Physical examination showed red-violet skin lesions in the inferior right extremity. Ophthalmological examination showed a reduction ofthe visual fields and a bilateral papilledema. The rest of the examination was normal. A cranial magnetic resonance scan was performed (figures 1 and 2).