Atypical polypoid adenomyoma (APA) is considered as an uncommon benign uterine tumor. However, more and more cases show that it has characteristics similar to malignant tumor, such as high recurrence and malignant transformation. There is no consensus on the standard treatment for APA now. Total hysterectomy is the main treatment modality for APA patients, but a conservative treatment can be performed in young patients who wish to preserve their fertility. This article aims to summarize the current knowledge about APA from several aspects, which may provide theoretical basis for the clinical treatment of this rare disease in the future.