This is a rare case of struma ovarii combined with sarcomatoid carcinoma. Whether it's struma ovarii or ovarian sarcomatoid carcinoma, the incidence of both is extremely low and rarely reported, so there are no clear guidelines for either disease and to our knowledge this may be the first case of combined occurrence of both. Therefore, this report describes its clinical manifestations, diagnosis and treatment, analyzes the pathogenesis, and summarizes the previous literature in the hope that it can be helpful to other tumor-related medical personnel and provide material support for the formation of guidelines in this disease.