BACKGROUND Inflammatory myofibroblastic tumor (IMT) is a rare disease, and uterine IMT is even rarer. IMT is hard to distinguish from endometrial polyp and submucous myoma. The treatment of IMT is still controversial. Here, we report a case of uterine IMT, discussing both pathological and therapeutic aspects. CASE REPORT A 32-year-old woman was admitted to our hospital for a uterine mass, hypermenorrhea, and anemia. She had been suffering from these symptoms for almost a year. Pelvic ultrasound and MRI revealed a mass about 7 cm in diameter at the bottom of the uterus. Serum tumor markers were negative. She was diagnosed with submucous fibroids of the uterus. Then she underwent hysteroscopic mass resection. Histopathological and immunohistochemistry stain analysis revealed IMT of the uterus. Due to the malignant potential of IMT, she was advised to undergo a total hysterectomy, but she refused because she wanted to retain the uterus and fertility. A watch-and-wait strategy without any therapy was chosen, and the patient is currently disease-free after 18-month follow-up. CONCLUSIONS IMT is a disease with malignant potential and may recur at a late stage; hence, a correct diagnosis is essential for patients with IMT. Surgery is the preferred treatment for IMT. For early-stage, young women who want to preserve fertility, conservative surgery is acceptable, but close follow-up is required to avoid recurrence and metastasis. If a patient cannot undergo surgery or the disease has metastasized extensively, targeted therapy for ALK gene, immunotherapy, and other methods can be considered.