The risk of the development of second primary malignancies is increased in patients with head and neck squamous cell carcinoma compared with the general population. Furthermore, this increased risk largely consists of aerodigestive tract malignancies, including head and neck cancer, lung cancer and oesophageal cancer. The World Health Organization classification defines two main categories of natural killer (NK) cell-derived neoplasms: extranodal NK/T-cell lymphoma (ENKTL), nasal type, and aggressive NK-cell leukaemia. Extranodal NK/T-cell lymphoma, nasal type, formerly called angiocentric lymphoma, is the most common cause of the syndrome known as “lethal midline granuloma”. Herein we aimed to report the development of ENKTL nasal type in a patient who received curative chemoradiotherapy for laryngeal epidermoid carcinoma and obtained complete remission.