Lymphoblastic lymphoma (LBL) is a neoplasm of lymphoid precursors pres
enting usually as acute leukemia with bone marrow and peripheral blood
involvement. Primary cutaneous involvement of LBL with a pre-B phenot
ype has to be considered an extremely uncommon occurrence, accounting
for less than 1% of all non-Hodgkin lymphomas. A child with an LBL inv
olving a single cutaneous manifestation of 6 months duration is presen
ted. At the time of presentation, the lesion consisted of a rapidly en
larging deeply infiltrated tumor on the upper arm. Immunophenotypic an
alysis performed an paraffin-embedded and frozen tissue sections revea
led 2 pre-B phenotype of the tumor cells. Similar results were obtaine
d from lymph node and bone marrow biopsy specimens. After 26 months of
polychemotherapy, the patient is currently in complete remission. We
wish to add this case to the current literature of LBL with cutaneous
involvement, emphasizing the importance of a correct diagnosis and the
excellent response to the therapeutic regimen.