Both sarcoidosis and primary Sjögren syndrome (pSS) are multisystem disorders of unknown etiology, which share certain clinical features, making the differential diagnosis a real challenge in clinical practice. Several published case reports and case series have posed the question as to whether there is a real association - mimicry between the two diseases or it is just coincidence. We attempt, after systematic and comprehensive research of the relevant published literature, to present all those data, clinical or paraclinical, which could be useful in the diagnostic approach and the distinguishment of the two diseases. It is certain that, besides the classic diagnostic methods, emerging is the role of immunology and genetics on this direction, although not established yet.