Malignant melanoma arises from melanocyte, which has the ability to synthesize the pigment melanin. Melanocytes are derivations of neural crest cells and are widely distributed throughout all cutaneous and mucosal surfaces. Overall, 20% of all malignant melanomas arise in the head and neck, of which only 10% affect the mucous membranes. Less than 1% of all malignant melanomas affect the nasal cavity. The authors have recently experienced a case of primary malignant melanoma originated in the left nasal septum and extended to the nasal floor, and the patient had been treated by wide excision of the tumor with extended hemitransfixion and degloving approach and postoperative irradiation. We report a case of primary malignant melanoma on the nasal septum of 54 years old male patient with review of literature.