Childhood osteoporosis is an emerging health issue with ramifications that can persist into adult life. Osteoporosis can result from a genetic disorder (primary) or be secondary to another disease or treatment (secondary). It is defined by the presence of low trauma vertebral compression fractures or a combination of low bone mineral density and a clinically significant fracture history. All children, especially those with risk factors for secondary osteoporosis, should have their bone health monitored and optimised. Investigations such as dual X-ray absorptiometry, peripheral quantitative computed tomography and spinal radiographs can aid diagnosis and monitor disease progression. Bisphosphonate therapy remains the mainstay of medical therapy but new therapies are emerging as more is understood about the mechanisms regulating bone development. Advances in genetic testing will improve our understanding and possibly management of primary childhood osteoporosis.