Myxoma is a rare mesenchymal tumor that is especially uncommon in the head and neck. We report a case of a myxoma of the larynx in a 70-year-old man, which, to our knowledge, is the second case reported in the English-language literature. Although benign, myxomas have a propensity for local recurrence due to their tendency to infiltrate surrounding tissues. They commonly appear encapsulated, but simple enucleation is not adequate treatment; excision should include a margin of normal tissue around the tumor.