Malignant melanoma within the gastrointestinal tract is an uncommon neoplasm that is usually metastatic in origin, with primary melanomas being relatively uncommon. Embryologically melanocytes normally exist in the esophagus, stomach, small bowel, and anorectum and this theory supports the primary melanoma of the gastrointestinal tract that has been confirmed for lesions occurring through several published reports. However, most patients with brain metastases from malignant melanoma are diagnosed after treatment for known extracranial metastases and have poor outcomes. Our case is unique in that we discuss an unusual case of 69-year-old female patient presented with unsteady gait as the first symptom of disease and where the presumed primary lesion later was found in the terminal ileum on colonoscopy. Treatment consisted of surgical removal of the terminal ileal lesion with chemotherapy, whole-brain radiotherapy, and cyberknife radiosurgical procedure. Patient was in remission for more than 14 months and later succumbed to disease. Despite the advances in therapeutic options, prognosis for patients with melanoma brain metastases remains poor with a median survival time of six months after diagnosis.