Two cases of Rosai-Dorfman disease with polypoid nasal infiltration mimicking nasal tuberculosis and malignant lymphoma are reported. This rare benign disease was first described by Rosai and Dorfman in 1969 and is characterized by histiocytic proliferation. It is seldom considered in the differential diagnosis of granulomatous diseases due to its rarity and histological similarity to other diseases. Extranodal manifestations of this disease are uncommon. Although no specific treatment can guarantee a sustained remission of this disease, surgery for loco-regional lesions can result in long-term symptomatic control and restoration of function. Both patients underwent endoscopic resection of the nasal polypoid lesions and have subsequently been free of recurrence. Loco-regional infiltration of the nasal cavity by Rosai-Dorfman disease is effectively managed by endoscopic resection.