Laryngeal spindle cell neoplasms are uncommon tumors, frequently misdiagnosed as reactive lesions or mesenchymal malignancies. One of the rare mesenchymal tumors of the larynx is myxoid spindle cell tumor (angiomyxoma), a benign proliferative lesion. It is considered the laryngeal counterpart of the aggressive angiomyxoma of the pelvis and perineum. Although benign, angiomyxomas have a propensity for local recurrence due to their tendency to infiltrate surrounding tissues. They commonly appear encapsulated, but simple enucleation does not provide adequate treatment; excision should include a margin of normal tissue around the tumor. We describe a case of laryngeal angiomyxoma in a 40-year-old man that, to the best of our knowledge, is only the second such case reported in the literature. The tumor in our case showed no histologic evidence of aggressive behavior, but because of the potential for local recurrence, close follow-up was warranted.