Epithelioid trophoblastic tumors are exceptionally rare malignant forms of gestational trophoblastic neoplasia arising from the placenta. We present here a case of a 33-year-old woman with an epithelioid trophoblastic tumor with metastatic disease to the lungs that was initially thought to represent cervical carcinoma. After appropriate diagnosis and treatment, the tumor responded to therapy and was in remission. The cancer recurred 5 years later with new lung metastases. 18F-FDG PET/CT was used for initial tumor staging, to evaluate treatment response, and to help detect the recurrence.