Desmoplastic melanoma is a rare melanoma subtype, accounting for 1%–4% of all melanomas, with distinct clinical, histopathological, and molecular features. It predominantly arises in chronically sun-damaged skin, particularly in older, fair-skinned individuals, and is associated with a high mutation burden and ultraviolet radiation signature. Subclassified into “pure” and “mixed” types, desmoplastic melanoma exhibits prognostic differences, with “pure” desmoplastic melanoma demonstrating better outcomes. Clinically, desmoplastic melanoma often presents as amelanotic, scar-like lesions, leading to diagnostic delays. Histologically, it is a dermal-based spindle cell tumor with dense collagenous stroma, often requiring immunohistochemistry for confirmation. Molecularly, desmoplastic melanoma is characterized by NF1 truncating mutations and other alterations in tumor suppressor genes. Treatment typically involves wide local excision, with adjuvant radiotherapy for high-risk cases. Understanding desmoplastic melanoma’s unique features is critical for accurate diagnosis and optimized management.