Molecular and genetic analyses of tumor cell show that cellular oncogenes and suppressor genes are involved in neoplastic transformation. In pediatric tumors oncogenes as N-myc play an important role in the tumor progression. In retinoblastoma, neuroblastoma, Wilms' tumor, and rhabdomyosarcoma loss of heterozygosity for specific chromosome loci has been suggested to be a critical step in cancer development. Oncogene abnormalities can also be useful as a molecular tumor factor to foresee the prognosis of the disease. The present article is a review on the role of the oncogenes and suppressor genes in pediatric solid tumors.