BackgroundRosai–Dorfman disease is a rare benign histiocytic disorder characterized in most cases by painless cervical adenopathy. Less than 10% of extranodal cases involve bony lesions. Primary bone Rosai–Dorfman disease in the absence of nodal disease is extremely rare.Case presentationA 48 year-old Caucasian male presented with progressive right-sided otalgia, tinnitus, vertigo, and hearing loss. A right temporal bone lytic lesion was detected on diagnostic imaging. Resection of the lesion and histopathological examination revealed Rosai–Dorfman disease.ConclusionsRosai–Dorfman disease primary bone lesions are an atypical presentation of a rare disease. This is the second reported case of Rosai–Dorfman disease arising within the temporal bone. This case study reveals that Rosai–Dorfman disease should be considered for patients presenting with inflammatory/lytic lesions of the temporal bone, in cases where infection and malignancy have been excluded.