Acral lentiginous melanoma (ALM) is a rare subtype of cutaneous malignant melanoma not linked to UV exposure that carries a poor survival prognosis. ALM is defined by its acral location, involving the palms, soles, and subungual regions of the hands and feet, as well as by its unique clinical characteristics. We have reviewed the incidence, diagnostic standards, histopathology, dermatoscopic features, reflectance confocal microscopy features, genomic alterations, and treatment of ALM. We highlight several recent case reports that showcase the diagnostic challenge that ALM poses and the barriers to arriving at a prompt and accurate ALM diagnosis. Increased patient and provider education, empowering early detection and treatment, as well as the development of new more targeted therapeutics are critical to improving ALM outcomes.