A 70-year-old woman with pulmonary carcinomatous lymphangitis and paraaortic lymph node metastases due to gastric cancer, was treated by combination chemotherapy of S-1 and irinotecan (CPT-11). After one course of the chemotherapy, pulmonary carcinomatous lymphangitis and paraaortic lymph node metastases were remarkably improved. Diet intake was improving and cancer pain remarkably declined. Because the origin of gastric cancer was not improved, total gastrectomy, distal pancreatectomy and splenectomy were performed. After surgery, relapse of pulmonary carcinomatous lymphangitis caused death of the patient. The combination chemotherapy of S-1 and CPT-11 was effective for pulmonary carcinomatous lymphangitis and paraaortic lymph nodes metastases due to gastric cancer. However, careful consideration is required since surgery is performed on a patient who had suffered pulmonary carcinomatous lymphangitis.