Introduction: Congenital anomalies of female genital tract in children and adolescents are more commonly detected in the age of menarche with several symptoms caused by obstruction. These diseases are rare and able to cause a number of serious complications, if early diagnosis and treatment were not performed. This article is aimed to review a modern approach to the diagnosis and treatment of these diseases and also to highlight some aspects that require further investigations. Materials and methods: A literature search was conducted using the following databases: PubMed, Goоgle Scholar, e-library, international guidelines and clinical recommendations. The key words we have used for our search: «congenital malformations», «distal vaginal atresia», «imperforated hymen», «OHVIRA», «transverse vaginal septum», «hematocolpos», «obstructive uterovaginal anomalies», «surgical treatment in pediatric». International clinical studies we have found were analyzed and included in this review. Results: We have explored the current literature related to the topics about a number of obstructive malformations, such as a distal vaginal atresia, transverse vaginal septum, imperforated hymen and obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). Conclusion: The main conclusions we have made are the need for studies with a larger sample of patients and longer follow-up for analyzing the long-term outcomes and possible complications during the postoperative period. Another unresolved question is the recommended age for conducting a surgical treatment and also there is a need to examine possibility of creating new algorithms for an earlier diagnosis.