Tumors of histiocytic or dendritic cell origin appear to occur with increased frequency in patients with lymphoma. Recent molecular data have demonstrated clonal relationships between the lymphoma and the histiocytic/dendritic cell tumor in some of these cases. Clinical, pathologic, and experimental data suggest that this phenomenon probably represents transdifferentiation of the lymphoma clone to a histiocytic/dendritic cell lineage in most cases. Awareness of this entity is necessary to prompt comparative molecular studies in appropriate cases.