Perivascular epithelioid tumor (PEComa), a recently defined tumor, is a very rare disease affecting various organs, most often the uterus. This tumor displays a variety of histologic and clinical features and at this point is regarded as a tumor with uncertain malignant potential. A 9-year-old girl with abdominal pain and vaginal spotting was diagnosed with PEComa of the uterus with metastasis. She received chemotherapy comprising vincristine, ifosfamide, and doxorubicin, as well as radiotherapy after surgery. After this multimodal treatment, there was no evidence of recurrence or further metastasis. She remains disease-free 1.5 years after her initial diagnosis. PEComa of the uterus displaying malignant characteristics may have a more favorable response to more aggressive therapy.