We report a case of bilateral lacrimal gland involvement as the first sign of Waldenstrom's macroglobulinaemia in a patient with sarcoidosis. Histological analysis of an incisional biopsy revealed a lymphoplasmocytic lymphoma consistent with Waldenstrom's macroglobulinaemia. No noncaseating granulomas were encountered. Systemic treatment was initiated and resulted in complete resolution of the lesions. In a patient with a systemic disease, such as sarcoidosis, and lacrimal gland involvement, a biopsy of the lacrimal gland mass should be taken to make a correct diagnosis and start appropriate treatment.