Marginal zone lymphoma (MZL) is an indolent neoplasm of mature B cells, classified by the World Health Organization into three categories: nodal, splenic, and extranodal MZL of mucosa-associated lymphoid tissue (MALT lymphoma). We present an unusual case of MZL with cutaneous, leukemic, and bone marrow involvement at presentation and expression of an aberrant myeloid-monocytic phenotype. This case is best classified as MZL of leukemic subtype.