Rosai-Dorfman disease (RDD) is an uncommon non-neoplastic lymphoproliferative disorder. It usually manifests as bilateral, painless, massive cervical lymph node enlargement in children and young adults, hence the name sinus histiocytosis with massive lymphadenopathy. Extranodal involvement is observed in 25 to 40% of cases. Involvement of nasal cavity is rare and those presenting with renal failure are exceptional. We report a rare case of 16-year-old boy who was diagnosed as a case of RDD of cervical lymph node before 6 years, now presenting with nodular lesions in the nasal cavity and features of renal failure. The nasal lesion was confirmed to be RDD histologically.