Extranodal NK/T-cell lymphoma, nasal type (ENKTCL), previously known as lethal midline granuloma is a distinct clinico-pathological entity associated with Epstein-Barr virus that typically causes destruction of the midface, palatal and orbital walls. In addition, ENKTCL can involve the skin, soft tissue, testes, gastrointestinal and upper respiratory tract. ENKTCL neoplastic cells express some T-cell associated antigens, most commonly CD2 and cytoplasmic CD3epsilon and, in favour of an NK-cell origin, CD56. Early stage disease may respond to radiotherapy alone, however late stage disease does not respond well to any available therapies. Overall, patients with ENKTCL have a cumulative probability of survival at 5 years ranging from 37.9% to 45.3%.