Although severely low bone density is relatively rare in the pediatric population, it can be a significant problem in many patients with chronic illness. As peak bone formation occurs during adolescence, it is crucial that pediatricians and other care providers for this patient population recognize the significance of attainment of adequate bone. Dietary intake of vitamin D and calcium should be optimized, and correction of underlying causes of poor bone density should occur whenever possible. Assessment of bone density is difficult, as each technology available has problems, and none of the technologies are well-associated with fracture risk in pediatric patients. Once diagnosis of severely low bone density is established, treatment options are limited and poorly studied. The benefits of bisphosphonate therapy appear to outweigh the risks in patients with low bone density and frequent fragility fractures, and it appears that most improvement with bisphosphonates occurs within the first 2 to 4 years. Evidence, however, is emerging that once off therapy, bone turnover remains decreased for at least several years. During that time, improvements in bone density are decreased. Many questions remain regarding duration of therapy with bisphosphonate therapy and the long-term effects on the children who receive this medication. Anabolic therapies may become important in the future, but there is currently extremely limited information regarding their use in pediatrics.