Aim: To study the effects of growth hormone (GH) deficiency (GHD) and GH replacement therapy (GHRx) on bone mineral density (BMD) and body composition. Methods: 59 GHD children participated (age range 0.4–16.9 years); the follow-up period was 6 years. Lumbar spine BMD (BMD<sub>LS</sub>), total-body BMD (BMD<sub>TB</sub>), and body composition were measured prospectively using dual-energy X-ray absorptiometry. Results: Mean BMD<sub>LS </sub>and BMD<sub>TB</sub> were significantly reduced at the time of the diagnosis. The bone mineral apparent density of the lumbar spine (BMAD<sub>LS</sub>) was reduced to a lesser degree. The BMAD<sub>LS</sub> increased to normal values after 1 year; BMD<sub>LS</sub> and BMD<sub>TB</sub> normalized 1 year later. At the time of the diagnosis, the lean body mass was reduced and steadily increased during GHRx. Percentage of body fat was increased at baseline and normalized within 6 months. The severity of GHD was not associated with the BMD at diagnosis or the response to GHRx. Conclusion: Areal BMD<sub>LS</sub> and BMD<sub>TB</sub> and, to a lesser extent, BMAD<sub>LS</sub> are decreased in GHD children, but normalize within 1–2 years.