Objective To analyse clinical characteristics, diagnosis and treatment of carcinoma showing thymuslike differentiation. Methods The clinical data of a case of carcinoma showing thymuslike differentiation treated in our department was retrospectively analyzed and literature was reviewed．The clinical characteristics, diagnosis diagnosis and treatment of this rare disease were summarized．Results We find the diagnosis of carcinoma showing thymuslike differentiation must rely on immunohistochemical staining, and surgery is the major treatment. Conclusion Understanding clinical characteristics, diagnosis and treatment of carcinoma showing thymuslike differentiation can be very important to improve the ability of differential diagnosis and standardization of treatment.