SummaryThe«largc cell anaplastic lymphoma, Ki–1 posili ve»is a recently described lymphoma subtype (about 1–8% of all NHL).Distinction from Hodgkin’s disease and true histiocytic lymphoma/malignant histiocytosis is not always possible, even by experienced pathologists. It was recently incorporated in the updated Kiel Classification of lymphomas. Classical histologic appearance is a sinusoidal growth pattern in lymph nodes and presence of large bizarre anaplastic cells. Use of cellmarkers LCA, EM A and Ki-1 or Ber-H2 is essential for diagnosis.The mean age of patients is 50 years. Approximately 50% of patients have an advanced stage (III-IV). Prognosis depends on age and tumor localisations. Cutaneous involvement only is usually associated with a good prognosis. Median survival for patients with extra-cutaneous disease is 13 months. Treatment with intensive chemotherapy is usually needed. Long term remissions are more frequently seen in children and adolescents.