Extracutaneous melanomas (ECMs) represent a heterogeneous group of melanoma subtypes characterized by distinct clinical and biological features from cutaneous melanoma. These subtypes share an aggressive natural history with high mortalities compared with nonacral cutaneous melanoma (NACM). Although recent advances in NACM have made significant improvements in morbidity and mortality, ECMs continue to lag behind. As the pathogenesis and molecular features of these rare subtypes continue to emerge, therapeutic research has aimed to closing the gap.