This study tries to define the incidence, survival, and risk factors of childhood cancer in Iran. A literature review on published articles was conducted by using different search engine from November 1974 to October 2008. The incidence rate of childhood cancer in Iran was 48 to 112 and 51 to 144 per million among girls and boys in multi geographical settings, respectively. The most common cancer in children from 0 to 14 years old were leukemia (incidence rate=8 to 62/million), lymphoma (3 to 23), and central nervous system tumors (3 to 22). The mortality rate of childhood cancers was 42 and 49 per million among girls and boys, respectively in 2004. A positive association between ABO blood groups, family history of cancer, drug usage during pregnancy, fathers' job, history of radiation, and parental smoking habit were found with childhood leukemia. It is essential that the effect of geographical differences on childhood cancer incidence rates and high mortality rate to be investigated in future studies.