An unusual case of variant HCL with multiple ribosomal lamellar complexes and complex chromosomal changes is described. The karyotype of the main cell clone is characterized by involvement of chromosome 5 at q13.3 and interstitial deletion of 7q. However, there is no other evidence of myelodysplasia and the abnormal cells are of lymphoid origin with a B-cell immunophenotype. The clonal chromosomal evolution involves rearrangements at sites known to be specifically altered in lymphoid tumors.