Introduction: Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is a rare benign histiocytic disease of unknown etiology, usually associated with lymph node enlargement in various superficial or deep sites.It usually shows a prolonged clinical course with occasional exacerbation and remission phases. In this article case of RDD in a 5-year-old male with large right inguinal and bilateral cervical lymphadenopathies were, occasional fever and anemia was described. The clinical presentation, histopathologic characteristics, and treatments of RDD were discussed.