Objective To discuss the diagnosis, treatment, and follow-up visit mode of thyroid carcinoma showing thymus-like elements (CASTLE). Mothods For a systematic review of a case with CASTLE, the domestic and overseas literature was searched to analyze final diagnosis, treatment and follow-up visit indexes of that case. Results For CASTLE, the pathology combined with immunohistochemistry was the only method for final diagnosis, the operation was the main treatment, and the ultrasound as well as the computed tomography (CT) could be the follow-up visiting indexes. Conclusion CASTLE is a special kind of thyroid carcinoma, which is different from differentiated thyroid carcinoma in diagnosis, treatment and follow-up visit.