Desmoplastic melanoma is a rare variant of malignant melanoma and one of the main causes of clinical and histological misdiagnosis in the realm of melanocytic neoplasms. Its relatively innocuous clinical appearance and the absence of pigmentation usually lead to a clinical misdiagnosis. The authors report a case of desmoplastic melanoma located in the lower lip of a 42-year-old man and review the literature regarding pathogenesis, clinicopathological features, differential diagnosis, and therapeutic management of desmoplastic melanomas.