We report a systematic literature review to identify (1) differences in body image (BI) between children and adolescents with cancer and healthy controls; (2) relationships between BI and demographic or medical variables; (3) implications of BI for psychological adjustment; and (4) relationship between BI and social support. Thirty-two studies were identified from computerized databases including BNI (1985-March, 2008), CINAHL (1982-March, 2008), MEDLINE (1950-March, 2008), PsychInfo (1806-March, 2008), and PubMed (1950-March, 2008). There was no consistent evidence regarding BI differences between children and adolescents with cancer and healthy controls. Relations between BI and gender, disease characteristics, and psychological adjustment were found. Changes in BI have adverse implications for self-esteem and adjustment, but can be moderated by social support. Future research should also adopt broader definitions of BI, and cancer-specific age-appropriate BI measures are needed to increase sensitivity of this work.