Résumé : We report here the case of a 7-month-old boy who developed anaplastic large cell lymphoma of true histiocytic origin or malignant histiocytosis, with fever, bone and bone marrow infiltration. Usual clinical features were absent. The neoplastic nature of the disease was supported by the presence of clonal chromosomal abnormalities [t(6;8)(p23;p21),der(8)del(8)(q11aq13), der(22) t(11;22) (q13;13)]. Neither B nor T lineage could be demonstrated here. Morphology, ultrastructural analysis, surface antigens expression, and cytogenetics were more specific for the monocyte-macrophage lineage.