Malignant melanoma is a highly lethal melanocytic neoplasm, usually affecting the skin. Primary malignant melanoma of the nasal cavity is rarely seen. Clinically, most patients display initial nonspecific symptoms of unilateral nasal obstruction or epistaxis. The prognosis is generally poor, with a mean survival time of 3.5 years. Extensive local invasion and distant metastasis to other organs may occur. The usual treatment of choice is radical excision. Radiotherapy and chemotherapy appear to have little effect. We report a fatal case of intranasal cavity malignant melanoma in which the patient initially presented with blood-tinged sputum, productive cough, and intermittent fever. Preoperative hepatic metastasis was found. Palliative surgery was performed to excise the nasal cavity tumor. Then, 6 courses of chemotherapy were further administered. Unfortunately, regional cervical nodal involvement and pancreatic head metastases occurred 1.5 years after the diagnosis. The patient's condition rapidly deteriorated, followed by death. We have chosen to discuss this aggressive condition because of its rarity and also to emphasize the importance of its early detection through vigilant attention to nonspecific nasal symptoms. A review of the literature concerning intranasal malignant melanoma is presented. We further discuss its possible etiology, site of origin, incidence, clinical presentations, principles of management, and outcome.