Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a newer pathological entity described in 1969. It is a self-limiting disorder of unknown aetiology. It is likely to be mistaken for lymphoma. It predominantly affects children and adolescents. However there are no publications on this disorder in the paediatric surgical literature. In this report we describe a 3-year-old girl who presented with this disease and we critically review the therapeutic options available for children. Prednisolone therapy with long-term follow-up appears to be sufficient. Surgery should be limited to biopsy and relief of compression symptoms.