Atypical polypoid adenomyoma (APA) is a rare benign uterine tumor, with less than 200 cases have been reported in English literature. Although, it is considered as a benign lesion and treated conservatively previously, more and more cases show that APA has a high rate of recurrence or residual, and is found to precede the development of carcinoma. Given the data from present research on APA, the therapy of APA becomes more complex and must be cautious, especially for the nulliparous and premenopausal patients. In addition, because of the low incidence, studies on this disease are less, and the etiology and pathogenesis of APA is still unclear. In this review, we aim to summarize recent researches concerning APA from multiple perspectives, including clinical presentation, histogenesis, immunohistochemistry and molecular features, diagnosis and differential diagnosis, treatment opinion and prognosis, which may provide theory and clinical basis for the future clinical treatment and research of this rare disease.