Medullary thyroid carcinoma (MTC) is a rare form of thyroid cancer that has different treatment approaches than differentiated thyroid cancer. MTC can be a challenge to diagnose by ultrasound imaging and fine-needle aspiration, so it should be considered in the differential diagnosis of any atypical thyroid nodules. Preoperative and postoperative serum calcitonin levels can help guide the surgical approach and help in monitoring and imaging. Calcitonin and carcinoembryonic antigen doubling times are helpful in prognosis and imaging. Rearranged during transfection (RET)-directed kinase inhibitors have significantly improved our therapeutic options for patients requiring systemic therapy and newer RET-inhibitors are being studied in clinical trials.