Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease)

Sinus histiocytosis with massive lymphadenopathy (SHML) is a distinct clinicopathological entity described by Rosai and Dorfman and differentiated by other childhood histiocytoses by its distinct characteristics. This is a rare pathology and should be kept in mind for differential diagnosis of neck masses, especially in childhood. In this article a case with SHML is presented and clinical features of the disease given.