Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare entity and a novel variant of the inflammatory myofibroblastic tumor (IMT). We report the occurrence and specific characteristics of EMIS in an adult woman. Eleven months after the operation, the patient had a recurrence and multiple metastases in the abdominal cavity. Since the tumor was spreading all over the abdominal cavity and ALK staining of the tumor was positive, crizotinib was suggested as adjuvant therapy. But she failed to respond to the crizotinib treatment and died of organ failure three months later.