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The case of a 24 year old man affected with Wiskott-Aldrich syndrome (WAS) is reported. The review of the charts of previous hospital admissions revealed the presence of an inherited trait and a long history of recurrent infections and hemorrhages. This young man was referred to our surgical department with the diagnosis of right lower limb gangrene, but the biopsy showed the lesion being due to cutaneous malignant lymphoma characterized by large polymorphic cells. All clinical and immunological elements typical of WAS were present: they are discussed with regard to the recent literature.
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