Extragonadal germ cell tumor with choriocarcinoma in males is a rare tumor. Usually, Choriocarcinoma occurs in the midline of the body, such as the retroperitoneum and mediastinum. The B-hCG is used for diagnosis and monitoring treatment response. It is an aggressive and highly metastatic tumor with poor outcome.A 24-year-old male patient was admitted in our hospital with abdominal pain that had progressively worsened over two months andwas associated with a weight loss of 7 Kg over 2 months. Patient had no significant medical, personal, and family histories.The ultrasound abdomen showed mild hepatosplenomegaly and hepatic hemangioma. A CT scan of the thorax and whole abdomen showed multiple bilateral lung lesions with areas of arterial phase hyperenhancement. The largest lesion measured 60 x 42 mm in the left basal lung, and abdominal images showed an 86 x 68 x 90 mm lobulated left para-aortic lesion in the infrarenal location.An excision of the right lower lobe lung with nodule was done, and the histopathological findings were consistent with those of a germ cell tumor with a component of choriocarcinoma.Tumor markers, particularly serum beta human chorionic gonadotropin (B-hCG) was significantly raised with level of 917950 mIU/mL. Other tumor markers done were Lactate dehydrogenase (LDH) - 725 U/L and Alpha feto protein (AFP) -0.762 IU/ml.Patient was diagnosed with a primary retroperitoneal choriocarcinoma with metastasis to lungs and was started on urgent inpatient chemotherapy.Extragonadal choriocarcinoma always has a late presentation when these bulky tumors cause compression symptoms and metastasize to other places. Diagnosis of extragonadal choriocarcinomas requires the exclusion of metastatsis from a primary tumor in the testes and distinguishing an extragonadal GCT from another poorly differentiated cancer via histopathological and immunohistochemical examination.