Pure primary NGCO (non-gestational choriocarcinoma of ovary) is a type of ovarian germ cell tumor with elevated human chorionic gonadotrophin (hCG), posing diagnostic challenges in the patients of reproductive age group. Clinically and histopathologically, NGCO is indistinguishable from GCO. The both are differentiated on the basis of DNA polymorphism analysis and presence of mRNA for BMG (β2-microglobulin) in NGCO. Diagnostic criteria set by Saito et al helps to make a diagnosis of NGCO. It is possible to cure NGCO while preserving fertility, which is an important consideration as most are young age group patients. As these are rare tumors, recommendations for treatment of primary nongestational choriocarcinomas are not available. The principles of surgical management of NGCO are similar to the ovarian epithelial tumors. GCO is treated by methotrexate based chemotherapy, but some studies reported that NGCO is resistant to this chemotherapy, and it requires more aggressive combination chemotherapy as later has bad prognosis as compared to GCO. Various chemotherapy regimens are BEP, EMA/CO, EMA/EP, VAC etc. The serial quantitative measurement of urinary or serum β-hCG is essential for diagnosis, monitoring efficacy of the treatment, and follow-up of the patients. Role of radiation therapy is limited as a palliative setting in metastatic NGCO. In this article, we have tried to conclude the diagnostic methods and best possible treatment protocol.