Medullary carcinoma accounts for only 1-5% of malignant thyroid tumors, making it very rare. Carcinoma arises from the malignant transformation of parafollicular cells that secrete the hormone calcitonin. There are two types of tumors - sporadic, which is more common (75%) and hereditary, which occurs as part of the MEN2 syndrome. Clinically, the tumor can be presented as a diffusely enlarged thyroid gland, enlarged solitary or multiple nodules, or shows signs of compression of nearby organs. Sometimes the first symptoms that appear are symptoms of metastases. World associations for thyroid diseases, such as ATA, ETA, ESMO, have published guidelines based on numerous studies to facilitate diagnostic and therapeutic decisions for clinicians. According to the mentioned guidelines, the diagnosis of medullary carcinoma is established by ultrasound, FNAC and measurement of the calcitonin level in the serum. It is also recommended to do genetic testing for everyone who is suspected of having MTC, because a large percentage of cancers are caused by mutations in the RET proto-oncogene. If metastases are present, they can be visualized by nuclear medicine imaging methods. 18F-DOPA-PET/CT is considered the best method for detection of MTC metastases. In the treatment guidelines, total thyroidectomy is the first choice for treatment of patients with locally advanced cancer, while systemic therapy, with drugs from tyrosine kinase inhibitors group, is indicated in patients with advanced disease. In case of advanced disease nuclear medicine therapeutic methods such as PRRT can also be offered, where somatostatin receptor agonists are labeled with beta emitters and thus destroy tumor tissue, but these methods are still being researched and will certainly be more prevalent in the future. Radiotherapy and chemotherapy as methods of choice for patients with MTC are still under investigation and are not recommended as first line in therapy.