BACKGROUND: Hurthle cell carcinoma represents approximately 3% of all differentiated thyroid cancers. The terminology is often confusing, and discrimination between Hurthle cell carcinoma and benign Hurthle cell tumors can be unclear. Thus, optimal treatment of patients with these diseases remains unsettled. METHODS: The authors reviewed published evidence on the presentation, biologic behavior, and treatment outcomes for this disease. In addition, they summarized their experience involving a series of 14 patients with Hurthle cell carcinoma. RESULTS: Hurthle cell carcinoma generally produces thyroglobulin and rarely takes up radioactive iodine. It is frequently bilateral or multifocal within the thyroid gland and often presents with local invasion. Hurthle cell carcinoma is associated with a high rate of locoregional recurrence and significant mortality. CONCLUSIONS: The authors advocate total thyroidectomy with central neck dissection as the therapy of choice for patients with Hurthle cell carcinoma.