Abstract: Primary extramedullary plasmacytoma (PEP) is an uncommon neoplasm of plasma cell origin which afflicts the head and neck mainly. In this study we report a rare case of a 34-year-old man who presented with left ear tinnitus, hearing loss, blocked feeling and headache. Exploratory tympanotomy revealed a mass extending into the attic and the mastoid antrum. Following canal wall-up mastoidectomy, the tumour was carefully removed. Histological examination (including immunoperoxidase staining) and thorough clinical, laboratory and radiological evaluation revealed an exclusively cytoplasmic monoclonal IgG immunoglobulin PEP. The combination of surgery (including a second-look procedure) and radiotherapy used in this case may be an over-treatment. However, the patient is still disease-free seven years after his first admission to hospital.