The aim was to systematically review and find evidence to determine when to extract deciduous teeth and place implants in young individuals with tooth agenesis. A search was made in MEDLINE on combinations of the terms 'tooth agenesis', 'deciduous teeth' and 'dental implants'. Publications with an abstract and written in the English language only were included. To give a background to the clinical management of young individuals with agenesis of teeth, publications on epidemiology of tooth agenesis, persistence of deciduous teeth, treatment outcomes after multi-disciplinary treatment planning and experiences of treatment with dental implants in young individuals were also reviewed. A search on the terms 'tooth agenesis', 'deciduous teeth' and 'dental implants' resulted in nine references and a search on 'dental implants' and 'tooth agenesis' gave 132 references; 46 met the inclusion criteria. Only two were prospective studies on treatment with implants in young individuals. A vast majority of publications on the clinical management of young individuals with tooth agenesis are reflections of clinical experiences and single case reports. Deciduous teeth are extracted for different reasons and at different ages in an optimal plan for a good treatment result from aesthetic and functional point of view. For ethical reasons, randomized clinical trials on when to extract deciduous teeth and place implants cannot be made in young individuals. There was only limited, low level evidence on when to extract deciduous teeth and place implants. Recommendations on treatment are based mainly on clinical experience.