Desmoplastic malignant melanoma (DMM) is an extremely rare subtype, encompassing up to 1% of all melanoma cases, worldwide. DMM features a prolonged clinical course and a variable clinical picture, which usually delays the exact diagnosis. The main differentiation from common malignant melanoma includes local recurrence rate, lymph node and distant organs involvement. DMM is further histologically divided into two subtypes – pure DMM and mixed / partial/ combined DMM. The division is important part of the diagnostic verification since these variants demonstrate different biological behavior and prognosis. Herein, a clinical case of a clinically atypical, pure desmoplastic malignant melanoma, occurring in a relatively young patient, is presented. A comprehensive overview highlights the most important DMM epidemiological, clinical and histological characteristics.