Subungual melanoma (SUM) is infrequent in the general population, accounting for 0.7-3.5% of all cutaneous melanomas. SUM absolute incidence is similar among different racial groups; however, the relative proportion among overall cutaneous melanoma cases within each population varies in relation to the frequency of sun-induced melanoma. Subungual melanoma most commonly presents as a discoloration of the nail, followed by a recalcitrant wound, a tumor, nail splitting and nail bed bleeding. In most cases, the clinical presentation will already exhibit features typical of late-stage lesions because many patients wait for several months or even years before consulting a physician for evaluation of nail changes. Misdiagnosis of SUM as subungual hematoma, chronic trauma or onychomycosis is still a frequent occurrence, significantly reducing the chances for early treatment. An appropriate diagnostic approach is crucial to allow early-stage diagnosis. The correct management of SUM hinges on early diagnosis and selection of the most appropriate surgical technique. Curative treatment of SUM currently entails surgical excision when the extent of invasion is limited.