Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a recently described, clinically aggressive variant of inflammatory myofibroblastic tumor with a predilection for intraabdominal sites, tendency to arise in male patients and resistance to conventional chemotherapy and radiotherapy. We present a case of a 15-year-old girl with EIMS of the ovary with a RANBP2-ALK fusion. During 2 years of follow up, she experienced multiple recurrences. Following classification of the tumor as EIMS, the patient began treatment with an ALK inhibitor (crizotinib), which led to disease improvement within weeks of administration. To the best of our knowledge, this represents the first reported case of EIMS arising in the female genital tract.