Intravascular large B-cell lymphoma, an extranodal large B-cell lymphoma, is a rare hematological malignancy with only a few reports of lung involvement. We report a case of intravascular large B-cell lymphoma with acute hypoxic respiratory failure and interstitial lung disease diagnosed via random skin biopsies. A 54-year-old woman presented with fever, cough, and dyspnea. Computed tomography imaging revealed findings concerning interstitial lung disease. The patient's respiratory status worsened despite the treatment with antibiotics and steroids. Generalized edema and thrombocytopenia also developed. Intravascular large B-cell lymphoma was clinically suspected and ultimately diagnosed by skin biopsy, although she had no apparent skin lesions. The patient's condition considerably improved after chemotherapy. Intravascular large B-cell lymphoma should be considered in patients with acute respiratory failure and interstitial lung lesions.