Introduction: Metastatic melanoma rarely metastasizes to the gastrointestinal tract (GIT). However, melanoma in the gastrointestinal tract is usually of metastatic origin, given that primary melanomas in this location are extremely uncommon. It is very important to differentiate between primary melanoma in the mucosa of the GIT and metastatic melanoma. Case Presentation: A 65-year-old woman with a history of primary cutaneous melanoma in the head region dating back to 2019 developed metastatic melanoma of the large intestine and terminal ileum after two years. Several months later, the patient died. Conclusion: The diagnosis of metastatic melanoma requires a multidisciplinary approach to determine the precise and definitive diagnosis as well as timely therapy, all with the aim of improving the quantity and quality of life of patients.