A thirty-year-old clerk presented with a history of left supraclavicular lymph node swelling of a month duration and newly detected multiple lung and mediastinal tumors. Abdominal computed tomography showed two retroperitoneal masses, but no abnormality in both testes was found by physical and ultrasonographic examination. Biopsy of supraclavicular lymph nodes revealed pure choriocarcinoma. Furthermore, beta-subunit of human chorionic gonadotropin was elevated up to 700 ng/ml but alfafetoprotein was normal, resulting in the diagnosis of primary retroperitoneal pure choriocarcinoma. Two cycles of combination chemotherapy with etoposide (VP-16), ifosfamide and cisplatin (VIP) was given and followed by bleomycin, etoposide and cisplatin (BEP) (2 cycles) because of cystitis symptom due to ifosfamide. Since post chemotherapeutic evaluation revealed only partial response, we surgically removed all residual masses in the lung, mediastinum and retroperitoneum, which histologically proved to be necrotic fibrous tissue. He has remained disease-free 15 months after surgery without additional therapy. In order to improve prognosis of primary retroperitoneal pure choriocarcinoma, intense chemotherapeutic regimen like VIP should be given, regularly repeated and followed by aggressive surgical resection of residual masses.