Acral melanoma (AM), including subungual melanoma (SUM), is a distinct form of melanoma primarily seen on glabrous skin, including palms, soles, phalanges, and nail apparatus. This subtype of melanoma is overall rare but relatively more common in patients of Asian, Hispanic, and African descent. These melanomas typically present as hyperkeratotic lesions or pigmented macules, which may go undiagnosed for an extended period due to their sometimes subtle presentation. The etiology of acral melanoma is multifactorial, with less emphasis on ultraviolet (UV) radiation and family history, and instead a possible association with trauma or stress. Molecular and genomic studies reveal some aberrations, such as gene amplifications in CCND1, CDK4, KIT, and NF1. Subungual melanomas appear to share similar pathogenic origins as acral melanoma, but show some differences such as a higher frequency of UV-related mutational signatures in subungual melanoma. Both acral and subungual melanomas demonstrate characteristic dermoscopic features. The final diagnosis is confirmed through histologic examination. Defining histopathological patterns, immunohistochemical features, and clinical information are imperative for a correct diagnosis of acral melanoma, given that the differential diagnosis includes benign entities such as acral lentiginous nevi, subungual nevi, and other entities such as acral fibromas and fungal melanonychia. Prognosis depends largely on Breslow thickness, ulceration, and lymph node status. Survival rates overall are reportedly lower than in other melanoma subtypes.