A 74-year-old man presented with a metastatic brain tumor in the right parietal lobe observed through an MRI scan. Lower gastrointestinal endoscopy revealed that the tumor was located in the rectum. He was diagnosed with Stage Ⅳb rectal cancer(cT4aN1bM1b[BRA, SKN]). After prior stereotactic radiotherapy for brain metastases, the patient underwent rectal amputation and D3 dissection as management for the primary tumor. His postoperative course was uneventful, and he was discharged from the hospital 33 days postoperatively. He displayed partial response with capecitabine plus L-OHP therapy, and chemotherapy was terminated due to the development of renal dysfunction. On follow-up, elevated tumor markers, enlarged left mediastinal lymph nodes, and FDG accumulation on PET-CT were observed. Despite initiating UFT/UZEL therapy, the patient was judged to have progressive disease. The patient was then administered 5-FU plus l-LV plus CPT-11. However, this was later discontinued due to the development of hyperammonemia. The patient was placed on follow-up observation due to the decrease in his tumor markers and the disappearance of his enlarged lymph nodes. He is still alive seven years after his initial diagnosis. We report a case of a patient with rectal cancer that metastasized to the brain and the skin. He was successfully managed with multidisciplinary therapy. A relevant literature discussion is also included.