Rosai-Dorfman Disease is a granulomatous disease of unknown etiology that is characterized by painless massive lymphadenopathy. It is also known as Sinus histiocytosis. Acute renal failure with sinus histiocytosis has been reported very rarely. In this article, we present a male patient who presented with hypercalcemia and acute renal failure. Bilateral, multiple, painless, palpable cervical lymphadenopathy was detected on physical examination. The high-resolution chest computed tomography revealed bilateral hilar lymphadenopathy. We discuss the disease by presenting this male patient whose excisional biopsy samples from the inguinal region were reported as Rosai-Dorfman disease.