Kosai-Dorfman Disease (RDD) is a rare, benign, generally sell-limiting clinicopathological entity with unknown etiology. Most of the cases are children and young adults with lymphadenopathy and extranodal involvement. Here we present a 4-year-old girl with cervical lymphadenopathy and orbital disease. RDD should be considered in the differential diagnosis of the benign and malignant disorders known to cause lymphadenopathy. The indications of treatment are also discussed.