A 50-year-old man presented with a 6-month history of localized alopecia on the left frontal area of the scalp. Biopsy revealed desmoplastic melanoma with associated neurotropism. Wide excision was performed and showed desmoplastic melanoma extending to excision margins. Hair follicles were focally reduced in number and were infiltrated by melanoma cells. He has subsequently required multiple wide excisions, exenteration of the left eye and radiotherapy. He has had multiple recurrences and has developed involvement of the first division of the trigeminal nerve intracranially. To the authors' knowledge, this is the first reported case of desmoplastic melanoma presenting as alopecia.