This review discusses the role of DICER1 gene mutations in thyroid diseases, highlighting their significance in both pediatric and adult thyroid nodules. DICER1, involved in RNA interference, is associated with various neoplastic and non-neoplastic conditions, including pleuropulmonary blastoma. Recent studies show that thyroid disease is the most common manifestation of DICER1 mutations, with thyroid follicular nodular disease and differentiated thyroid carcinomas being highly indicative of germline DICER1 mutations. Poorly differentiated thyroid carcinoma and thyroblastoma suggest somatic DICER1 mutations. Recognizing these manifestations is crucial for early genetic evaluation and risk classification for multi-organ malignancies. The study emphasizes the importance of identifying DICER1 mutations in thyroid nodules to guide clinical management and genetic testing. DICER1 mutations can lead to various thyroid neoplasms, including thyroblastoma and PDTC, which are specific to somatic mutations. The review also discusses the histopathological features of DICER1-related thyroid diseases and their implications for diagnosis and treatment. Overall, understanding DICER1 gene alterations is essential for the accurate diagnosis and management of thyroid diseases associated with DICER1 syndrome.This review discusses the role of DICER1 gene mutations in thyroid diseases, highlighting their significance in both pediatric and adult thyroid nodules. DICER1, involved in RNA interference, is associated with various neoplastic and non-neoplastic conditions, including pleuropulmonary blastoma. Recent studies show that thyroid disease is the most common manifestation of DICER1 mutations, with thyroid follicular nodular disease and differentiated thyroid carcinomas being highly indicative of germline DICER1 mutations. Poorly differentiated thyroid carcinoma and thyroblastoma suggest somatic DICER1 mutations. Recognizing these manifestations is crucial for early genetic evaluation and risk classification for multi-organ malignancies. The study emphasizes the importance of identifying DICER1 mutations in thyroid nodules to guide clinical management and genetic testing. DICER1 mutations can lead to various thyroid neoplasms, including thyroblastoma and PDTC, which are specific to somatic mutations. The review also discusses the histopathological features of DICER1-related thyroid diseases and their implications for diagnosis and treatment. Overall, understanding DICER1 gene alterations is essential for the accurate diagnosis and management of thyroid diseases associated with DICER1 syndrome.