Monocytic leukemia may be associated with specific cutaneous changes in about a third of all cases, most characteristically the plum-colored nodule. Nonspecific cutaneous xanthomas have occasionally been reported with a variety of leukemias, mostly in the pediatric literature, but the first report (to our knowledge) of acute myelomonocytic leukemia presenting with specific cutaneous xanthomatous lesions appeared in 1982. Herein is reported a case of chronic myelomonocytic leukemia presenting with a papular histiocytic eruption, and later showing striking cutaneous xanthomas, together with autopsy findings. Physicians should be aware of this rare but important association.