Intravascular large B-cell lymphoma (IVL) is a rare systemic disease characterized by clonal proliferation of neoplastic lymphoid cells within the capillary lumina of small blood vessels. The most common sites of involvement are cerebrovascular or sinusoid areas, resulting in a wide variety of neurologic deficits. Herein we describe a case of IVL that presented with multiple internal and external organ involvements, including the skin, bilateral kidneys, and lung, and with an aggressive clinical course. The confirmative diagnosis was based on the microscopic findings of the skin lesion with a B-cell immunophenotype. The patient suffered from repeated severe dyspnea, hypoxemia, and high-grade fever with a simultaneous increase in serum lactate dehydrogenase and soluble IL-2 receptor levels. These manifestations responded dramatically to intravenous injection of corticosteroid (500 mg/day for 3 days). Despite various clinical presentations, no neurological abnormality was observed until the patient died 12 months after the initial combination chemotherapy.