Coagulation factor VII (FVII) deficiency (OMIM 227500) is the most common of the rare hereditary coagulation disorders characterized by autosomal recessive inheritance with variable penetrance. The estimated incidence of coagulation factor VII deficiency is 1:500,000. Clinical heterogeneity is a special feature of this coagulation disorder, the severity of which ranges from severe to mild or even asymptomatic forms. In the study were included three unrelated pediatric patients (3 female) with confirmed factor VII deficiency and members of their families (2 male and 2 female) Genetic analysis was carried out by next generation sequencing using Nextera XT, examining all exon and the exon/intron boundaries of F7 gene using a genetic analyzer MiSeq (Illumina, USA). All clinically significant observations were confirmed by Sanger sequencing. We identified the pathogenic variant in all pediatric patients with factor VII deficiency in F7 gene. Four unique variants were identified: two missense mutation (c.1061C>T, p.Ala354Val; c.283G>A, p.Glu95Lys), one frameshift variant (c.1391delC, p.Pro464His fsTer32), one point mutation of proximal promoter region (c.1–32A>C). Genetic analysis indicated that patient 1 had heterozygous compound of maternal missense mutation c.1061 C > T and frameshift variant c.1391delC and paternal point mutation of proximal promoter region c.1–32A>C. Patient 2 was identified homozygous of two mutation c.1061C > T and c.1391delC, while her both parents were heterozygous of this mutations. In patient 3 was detected heterozygous compound of missense mutation c.1061C>Tand missense mutation c.283G > A. DNA samples of parents of patient 3 was unavailable for genetic analysis. All these variants have been previously reported in the EAHAD F7 databases, except for the missense variant c.283G > A, p. Glu95Lys, which is classified as variant of uncertain significance. We have performed genetic analysis for the identification of pathogenic variants in coagulation factor VII deficiency patients for the first time in Belarus. Genetic analysis of coagulation factor VII deficiency patients is useful to confirm diagnosis, to determine the carrier status and genetic counseling.