Rosai–Dorfman disease (RDD) also known as histiocytosis with lymphadenopathy is uncommon, often self-limiting benign, not– Langerhans-cell histiocytic proliferative disorder. It usually affects young adults and children, with no predilection for sex. More than 80% of patients present with painless cervical lymphadenopathy, with or without fever. Other presentation sites include upper aero-digestive tract, orbits, and the paranasal sinuses. Extra nodal involvement can occur from 20% to more than 40% of cases, and common sites of involvement include the skin, upper airway, soft tissue, bones kidney, lower respiratory tract, liver, orbit and central nervous system. Treatment is not necessary for most instances, but some patients, when symptomatic, may require medical intervention. There is no established guideline for the management of this condition and various therapeutic modalities are used, including surgery, radiotherapy and or chemotherapy.