This study aimed to systematically review literature on the association between birth weight and pediatric cancer risk. Sixteen studies were qualitatively synthesized and seven were quantitatively synthesized. The results found an overall direct correlation between higher birth weight (≥2500g) and higher childhood cancer risk, while lower birth weight (<2500g) was associated with lower risk. However, for acute lymphocytic leukemia specifically, risk was higher for birth weights under 2500g. Significant heterogeneity between studies means these findings should be interpreted cautiously. Prospective cohort studies are needed to better understand the impact of prematurity on pediatric cancer development.