Özet:

Rosai-Dorfman Disease (RDH) is an unknown, often self-restricted clinico-patological picture of a rare benign disease. The majority of cases are presented by children and young adults who apply with lymphadenopathy and extranodal region constipation. In this article, the disease was discussed by presenting a four-year-old girl who was orbited with cervical lymphadenopathy. RDH should be considered in the distinctive diagnosis of other benign and malign diseases that lead to lymphadenopathy. Rosai-Dorfman Disease with Extranodal Involvement: A Case Report and Discussion Rosai-Dorfman Disease (RDD) is a rare, benign, generally self-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.