This review focuses on both hereditary medullary thyroid cancer (MTC) and hereditary nonmedullary thyroid cancer (NMTC) and discusses the genetics, clinical diagnosis and evaluation, and surgical approach to treatment of these malignancies. Areas of innovation as well as areas of debate are highlighted and management recommendations are made. J. Surg. Oncol. 2015 111:51–60. © 2014 Wiley Periodicals, Inc.