X-linked lymphoproliferative syndrome (XLP) is an inherited immunodeficiency disorder characterized by one or more of three major phenotypic presentations. These presentations include a defective response to infection with the Epstein-Barr virus (EBV), acquired hypogammaglobulinemia, and malignant B-cell lymphoma (National Organization for Rare Disorders, 2001). Patients exhibit a range of symptoms, including life-threatening EBV infection, lymphoma or Hodgkins disease, suppressed bone marrow function with related immunodeficiency, aplastic anemia, and lymphohistiocytic disorder. In some cases, patients may have a proliferation of certain white blood cells (lymphocytes and histiocytes) in particular organs subsequent to an EBV infection. XLP can be associated with susceptibility to bruising and excessive bleeding because of a decrease in platelets (thrombocytopenia). The occurrence of a Burkitt type of malignant lymphoma in the ileocecal region should prompt for evaluation of XLP in the patient and family because it is a frequent finding in XLP.