Melanomas of the gastrointestinal (GI) tract are very rare, and their primary or secondary nature is often difficult to establish. We report the case of a 55-year-old Caucasian female who presented with an appendiceal melanoma. To our knowledge, appendiceal localization of melanoma has never been reported. We review the literature and discuss the issues relating to the origin of gastrointestinal melanomas. In conclusion, even if a primary site of melanoma was not evident in this case, the metastatic nature of this appendiceal melanoma cannot be completely ruled out.