Abstract: Objective To study the clinical characteristics, diagnosis and treatment of atypical polypoid adenomyoma (APA) of uterus. Methods Clinical data about 13 patients with APA of uterus admitted to our hospital from January 2006 to October 2011 were retrospectively analyzed. Results APA usually occurred in premenopausal women and mainly manifested as irregular menstrual bleeding and infertility. Of the 13 patients, 6 underwent total hysterectomy and were followed up for 67 months during which no abnormality was observed, 5 received hysteroscopic tumor resection and curettage, 3 was pathologically diagnosed with APA and received no further treatment, 2 was pathologically diagnosed with low malignant potential APA and treated with progestogen with relapse in 1 who was not pregnant after treatment. No malignancy was found in all patients. Conclusion APA is a benign tumor with a good prognosis. It may potentially become malignant and can be surgically removed with fertility reserved and treated with progestogen. APA patients should be regularly followed up. Surgery and hysteroscopy are recommended for its reexamination.