Primary mucosal malignant melanomas of the nose are rare, accounting for only 0.3 to 2% of all cases of malignant melanoma and about 4% of all head and neck melanomas. The amelanotic variant of mucosal malignant melanoma is even more rare, and the prognosis is poor. This variant usually arises in locations in which it is not noticeable, and therefore it is usually diagnosed at an advanced clinical stage when symptoms eventually manifest. We report a case of locally advanced amelanotic melanoma of the nasal cavity in a 55-year-old woman who presented with complaints of nasal obstruction and intermittent bleeding on the right side of the nose for 3 months and a gradually progressive diffuse swelling over the right periorbital and maxillary areas for 2 months with associated pain for 15 days. She was diagnosed with amelanotic melanoma on the basis of clinical, radiologic, and pathologic findings. In view of her advanced disease, she declined surgery and was treated with chemoradiotherapy, and she experienced a significant alleviation of her symptoms.