Sarcoidosis still remains a diagnosis of exclusion. It is unusual for the disease to be localised in the nose and sinuses and to manifest its symptoms in this site; the diagnosis in such cases is rather a difficult task. There is no symptom pathognomonic of the disease. A major role in the diagnosis of sarcoidosis is played by histologic evidence on the basis of which additional tests can be used. We present a case of primary sarcoidosis of the nose and sinuses which involved the orbit and had non-specific symptoms in the sinonasal region. The diagnosis of sarcoidosis was made ultimately only after decalcification of sample material taken from the ethmoidal labyrinth. Interspersed among the bone trabeculae there were the typical epithelioid cellular granulomas composed of epithelioid cells with round nuclei and prominent nucleoli, Langhans'-type giant cells, and a tender rim of chronic inflammatory infiltrate in the periphery. After initial beneficial response to the administered cortisone therapy (40 mg prednisolone daily for 6 months and then a daily maintenance dose of 10 mg) the control examination showed that the peri- and retrobulbar infiltrate persisted and the eye symptoms recurred. We therefore increased the dose and proceeded to a pulse therapy (120 mg of urbason daily and a daily maintenance dose of 30 mg). At present the patient is still receiving this therapy which has reduced as a result the local manifestations but Cushing's syndrome has developed as a side effect.