Fabry's disease is an X linked storage disorder caused by mutation of the gene for the lysosomal enzyme alpha-galactosidase A (alpha-GAL A). It is characterized by the accumulation of globotriaosylceramide (GL-3) in several organs and tissues with preferential involvement of the vascular endothelium. Symptoms include pain, angiokeratoma, corneal clouding, and damage to the heart and kidneys. The onset of haematological disorders has been described for some lysosomal storage disorders such as Gaucher but not for Fabry. Here we report the case of a Fabry patient who developed a small lymphocytic lymphoma discussing the relationship between these conditions and their management.