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Background . Fibular hemimelia (FH) is a congenital anomaly in which part or all of the fibula is hypoplastic, dysplastic, or absent, associated with a wide range of abnormalities of the affected limb. FH is a challenging problem in pediatric orthopedics. The aim of the study is to present the fundamentals of pathogenesis, diagnostic options, and surgical and conservative treatment approaches for children with FH, based on an analysis of scientific literature. Materials and methods . The search was performed in PubMed, Google Scholar and eLibrary.ru databases for publications using the following keywords: fibular hemimelia, fibular ectromelia, longitudinal ectromelia, congenital absence of the fibula, postaxial anomaly, and congenital longitudinal deficiency. Due to the rarity of this disease, the search depth was 70 years. Results and discussion . An analysis of the publications revealed that there is currently no consensus on the surgical orthopedic treatment strategy for children with FH. The main questions raised by orthopedic surgeons: to perform early amputation or reconstructive orthopedic interventions? when to begin reconstructive orthopedic treatment on the affected limb? at what age and how much should a limb be lengthened during the first lengthening? Conclusion . Currently, interdisciplinary care and age-appropriate management involving an obstetrician/gynecologist, orthopedist, psychologist, rehabilitation specialist, occupational and physical therapist, nursing staff, and orthotist/prosthetist are essential for the successful treatment of patients with FH. Reconstructive orthopedic treatment should be individualized, taking into account the severity of the anomaly, the patient’s age, and the family’s motivation and psychological readiness for treatment.