We present a case of systemic sarcoidosis in a 34-year-old woman initially presenting with bilateral and symmetric proptosis caused by lacrimal gland enlargement. Based upon clinical, biological and radiological findings, sarcoidosis was suspected with lacrymal gland, parotid and pulmonary lesions. Biopsy of enlarged lacrimal gland for histological examination revealed a non caseating granuloma compatible with the diagnosis of sarcoidosis. Sarcoid lesions regressed with corticosteroid therapy.