Methotrexate-associated lymphoproliferative disorder (MTX-LPD) is a lymphoma that develops during administration of methotrexate (MTX). So far MTX-LPD cases presented with colonic perforation have not been reported in Japan. A 69-year-old man who had been taking MTX orally for malignant rheumatoid arthritis presented to our hospital because of left lateral abdominal pain. An abdominal CT scan offered a diagnosis of colonic perforation. Emergency partial colectomy was performed. The resected colon involved an ulcerative lesion, and ulcerative perforation of MTX-LPD (diffuse large B-cell lymphoma) was diagnosed. He developed postoperative suture failure that required reoperation, but made a good recovery. There have been no signs of recurrence, as of 2 years after the operation. Out of 86 patients with MTX-LPD reported in Japan, 69% of them could go into remission with withdrawal of MTX and 33% of patients who received chemotherapy for unchanged disease, aggravation, or recurrence of the disease died. No significant difference was noted in the prognosis between those with and without latent EBV infection. As remission cases had been followed for an average of 20.4 months, long-term prognosis is obscure. In treating MTX-LPD patients like our case, it would be important to go to the trouble of creating stoma at the initial operation. Key words:methotrexate-associated lymphoproliferative disorder,MTX-LPD,colon perforation