ABSTRACT Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymph-adenopathy is characterized as massive, painless, bilateral, symmetric cervical lymphadenopathy, with fever, leucocytosis, elevated sedimentation rate, and hyper-gamma-globulinemia. This paper reports on the case of a 2-year-old child with history of progressive, bilateral cervical lymph-adenopathy associated with fever, generalized weakness. Histo-pathological and immune-histochemistry studies showed predominantly large histiocytes, emperipolesis and S-100 positivity suggestive of RDD. Patient presented with splenic abscess on follow up and managed conservatively. Here we are describing a 2-year old patient of RDD associated with splenic abscess, currently under strict follow-up.