Herein we present a 7-year-old β-thalassemia major patient who developed severe Henoch-Schönlein purpura (HSP) with renal, pulmonary involvement and invagination while under iron chelation with deferiprone. DNA analysis for familial Mediterranean fever revealed M696V mutation. Various cellular and humoral immunological impairments have been described in thalassemia major patients and the severe course of HSP in our case may be related to these underlying immunological defects. Am. J. Hematol., 2008. © 2007 Wiley-Liss, Inc.