Introduction:Sarcoidosis is a multisystem disease that commonly involves the lungs, but may also present with extrapulmonary manifestations. Genitourinary (GU) tract involvement has been traditionally thought to be rare, but that view may underestimate the true prevalence of the disease due to the often, silent presentation thereof.Methods:The literature pertaining to sarcoidosis from the general systemic point of view, etiology and therapy and with regard to specific organs was reviewed by identifying key words in a PubMed search. That material with special relevance to the Indian experience was emphasized.Results:There are a number of isolated case reports in the literature describing symptomatic and asymptomatic GU tract sarcoidosis. The world literature associated with the generalized syndrome was reviewed and summarized. Specific aspects of GU involvement are presented for each organ of the GU tract.Conclusions:It is critical for the practicing clinician to have a working knowledge of the clinical manifestations of this disease as it involves the GU tract, as well as to be able to distinguish it from tuberculosis and the various malignancies that affect this organ system.