Ovarian neoplasms in children and adolescents are rare pathologies (2.6–3.0 per 100,000 patients a year), however, they are sufficiently relevant in pediatric gynecology nowadays. The age of the patients highly correlates with different histological types of ovarian neoplasms. And this is a reason for discussing this disease in different age groups apart. Unfortunately, there are a number of unresolved issues connected with the etiology, pathogenesis and diagnosis. This paper discusses recent information related with the subject “ovarian neoplasms in pediatric gynecology” and particularly questions which require further study.The aim of the work is to conduct a review of recent literature which corresponds to this subject and to discover questions which require further study.The analysis revealed that the etiology and pathogenesis of a number of rare neoplasms has not been discovered fully yet and that can be the cause of the delayed disease diagnostics. Apart from this there are no tools for the preoperative stratification of the risk of malignancy and that can lead to more frequent radical operations for benign tumors.