Intravascular lymphoma (IVL) is a non-Hodgkin lymphoma in which atypical lymphocytes accumulate within small blood vessels. Patients most commonly present with neurologic and cutaneous findings; however, any organ system may be affected, which leads to difficulties in diagnosis. The objectives of this article are to review the current IVL literature and stress the importance of multiple skin biopsies in diagnosis. We also describe, to our knowledge, the first case of IVL treated with allogenic peripheral blood stem cell transplant (PBSCT). Intravascular lymphoma should be considered in the differential diagnosis of unexplained erythematous tender indurated plaques, nodules, and telangiectases. Single biopsy is not sufficient to rule out this entity. Intravascular lymphoma is a recalcitrant malignancy, and we describe a case that quickly recurred after treatment with cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab (CHOP-R), and necessitated allogenic bone marrow transplantation (BMT).