Medullary thyroid carcinoma (MTC) remains a rare endocrine tumour. 20-25% of MTC cases are genetically determined. The detection of the RET proto-oncogene mutation in 1993 led to an understanding of the unique genotype-phenotype relationships in hereditary medullary thyroid carcinoma (HMTC) and formed the basis for therapeutic decisions based on the molecular results. Currently, prophylactic thyroidectomy is a commonly adopted and accepted therapeutic method. The decision on the time and extent of surgery should be made based on the results of molecular examination, the assessment of calcitonin (Ct) concentration, and family history. Treatment of patients with HMTC requires the cooperation of a multidisciplinary team of experts and should be done in specialised centres only. The study is a review of the current guidelines for surgical management in MEN2 syndrome. (Endokrynol Pol 2019; 70 (4): 367-372)  368 Current surgical management in RET mutation carriers Agnieszka Czarniecka et al. Current surgical management in RET mutation carriers Agnieszka Czarniecka et al.