An unusual presentation of extranodal Rosai-Dorfman disease threatening the airway.

Rosai-Dorfman disease (RDD) is a rare benign systemic histiocytic proliferation, characterized by massive lymph node enlargement and sometimes associated with extranodal involvement. Even though it is considered as a benign disease, fatalities can occur due to its unusually large size and its location. Our case highlighting a primary extranodal site of vallecula, which is extremely rare and not reported in literature before. It presented with almost complete obstructing the oropharyngeal airway creating a life threatening situation, needed emergency tracheostomy.