Malignant melanomas in the head and neck region are uncommon. When they do occur, they usually represent a metastasis. To the best of our knowledge, only 11 cases of primary malignant melanoma of the external auditory canal have been previously reported in the English-language literature since 1954. We describe a new case, which occurred in a 72-year-old woman who presented with a large, lobulated, pigmented mass with ulcerated bleeding on its surface. The patient was scheduled for surgery, but during preoperative preparations she developed signs of rapid dissemination and metastases to the liver and lungs, and she died of multiple organ failure within 3 weeks of presentation. Apart from the rarity of malignant melanoma of the external auditory canal, this case included other extraordinary features that make it noteworthy. Our experience with this case underscores the importance of early diagnosis and prompt initiation of treatment for patients with this potentially fatal malignancy.