Over the last decade the discovery of fibroblast growth factor 23 (FGF23) and the progressive and ongoing clarification of its role in phosphate and mineral metabolism have led to expansion of the diagnostic spectrum of primary hypophosphatemic syndromes. This article focuses on the impairment of growth in these syndromes. Growth retardation is a common, but not constant, feature and it presents with large variability. As a result of the very low prevalence of other forms of primary hypophosphatemic syndromes, the description of longitudinal growth and the pathogenesis of its impairment have been mostly studied in X-linked hypophosphatemia (XLH) patients and in Hyp mice, the animal model of this disease. In general, children with XLH have short stature with greater shortness of lower limbs than trunk. Treatment with phosphate supplements and 1α vitamin D derivatives heals active lesions of rickets, but does not normalize growth of XLH patients. Patients might benefit from recombinant human growth hormone (rhGH) therapy, which may accelerate the growth rate without increasing body disproportion or correcting hypophosphatemia. These clinical data as well as research findings obtained in Hyp mice suggest that the pathogenesis of defective growth in XLH and other hypophosphatemic syndromes is not entirely dependent on the mineralization disorder and point to other effects of hypophosphatemia itself or FGF23 on the metabolism of bone and growth plate.