Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal large B-cell lymphoma that selectively affects small and medium-sized bloodvessels in the absence oflymph-adenopathy. The central nervous system (CNS) and skin are the organs most commonly affected. We describe the case of a 64-year-old male who presented to the emergency department (ED) complaining of asthenia and bilateral lower extremity edema that progressed rapidly to anasarca. On presentation, laboratory results were significant for elevated erythrocyte sedimentation rate (ESR) and lactate dehydrogenase (LDH) levels. A skin biopsywas performed, which revealed occlusion of blood vessels by atypical immunophenotype B lymphoid cells within the dermis and subcutaneous tissue. The immuno-histochemistry was consistent with IVLB CL. IVLBCL is an aggressive and rapidly fatal neoplasia with varied and nonspecific clinical manifestations, hence, a diagnostic challenge. This case shows an unusual presentation with asthenia and rapidly progressive edema.