We report the case of a patient with medullary thyroid carcinoma (MTC) who presented multiple pharyngoesophagolaryngeal metastases detected by flexible laryngoscopy. Pathology confirmed the diagnosis after resection with CO2 laser under direct laryngoscopy. Because of worsening dyspnea, complete circular pharyngolaryngectomy was performed with reconstruction of the pharyngeal region with a major pectoralis musculocutaneous flap. The postoperative period was satisfactory. At 17 months, calcitonin level was elevated; subcutaneous cervical metastases of the MTC developed and were resected. At 27 months, oral nutrition and phonation remained satisfactory. We discuss the diagnostic and therapeutic strategies used for this very unusual form of locoregional spread of medullary carcinoma of the thyroid gland.