Background and Objective: Considering the genetic heterogeneity of FMF and its diverse clinical manifestations, it is necessary to investigate the frequency distribution of heart valve involvement based on different MEFV genotypes. This study aims to fill the gap in knowledge about the cardiovascular burden of FMF in children, particularly focusing on how different genotypes affect the prevalence and type of valvular heart disease. Methods: In this study, a cross-section of 40 children under 16 years of age with Mediterranean fever, echocardiographic parameters of the patients: Myocardial performance index, pulmonary hypertension, ejection fraction, changes in heart valves, pericardial effusion and changes in the great vessels of the heart, left ventricle mass ,, was investigated. SPSS software version 27 was used for data analysis. Results: mild tricuspid regurgitation was observed in 2 cases (5%) of patients and mild pulmonary insufficiency was observed in 3 cases (7.5%) of patients. Analysis of available data showed that heart valve involvement has a significant and strong relationship with E148Q genotype (P=0.002 and r=0.635). Conclusion: Our study highlights a significant and strong association between E148Q genotype and heart valve involvement in children under 16 years of age with FMF. These findings emphasize the importance of genetic factors in the cardiovascular manifestations of FMF and the need for genotype-specific clinical management.