Floating-Harbor syndrome (FHS) is an extremely rare, genetically determined disorder characterized by intellectual disability, distinctive facial features, and short stature, combined with bone age delay and speech disorders. To date, just over 100 cases of patients with this syndrome have been reported in the literature. Our article describes the case of an 8-year-old boy diagnosed with FHS, who additionally suffers from primary pulmonary hypertension. In the article, we address whether the boy’s heart defect and cardiac conditions can be components of a genetic defect. In addition, we address the problem of growth deficiency in patients with FHS and present the results of treatment using recombinant human growth hormone. Given the extremely limited number of reported FHS cases, we believe our contribution will help expand the current understanding of the syndrome and further define its clinical phenotype.