An 80-year-old man with a history of diffuse large B-cell lymphoma (DLBCL) presented to the otolaryngology department due to hearing loss and fullness in the right ear. He was diagnosed with primary testicular DLBCL five years earlier, but complete remission was sustained after treatment. Computed tomography identified a soft-tissue mass in the right middle ear and mastoid cells, along with destruction of the petrous and sphenoid bones with soft-tissue mass and mucosal thickening in the maxillary sinus (Figure 1a-c). A weakly positive result was obtained for serum proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), at 5.2 IU/mL. The patient was referred to our department on suspicion of granulomatosis with polyangiitis (GPA) and otitis media with ANCA-associated vasculitis (OMAAV). He showed no other symptoms suggestive of systemic vasculitis, and serum soluble interleukin-2 receptor was slightly elevated to 643 U/mL (normal: <610 U/mL). The T2-weighted magnetic resonance imaging (MRI) showed signal hyperintensity in the right mastoid, suggesting mastoid effusion (Figure 2a). However, contrast-enhanced MRI revealed enhancement around the mastoid cells and cranial base and nodules in the maxillary sinus (Figure 2b, c). Diffuse large B-cell lymphoma was diagnosed from biopsied mucosa in the maxillary sinus, with no findings of granuloma or vasculitis.