Positron emission tomography/computed tomography (PET/CT) has become an important imaging modality in the non-invasive evaluation and monitoring of children with known or suspected malignant diseases. In sarcoma patients, [18F]FDG (FDG) PET and FDG PET/CT is useful in staging, therapy monitoring, and detection of relapse. However, FDG PET has been proven to be less sensitive than chest CT in the detection of pulmonary metastases derived from sarcoma. This disadvantage has been overcome using a PET/CT scanner. In neuroblastoma patients, PET using FDG is indicated in MIBG-negative cases. Furthermore, there are specific PET tracers for tumors of the sympathetic nervous system, such as [11C]Hydroxyephedrine (HED) and [18F]-labeled dihydrophenylalanine (F-DOPA), which can be used for PET/CT imaging for detection of disease, staging and monitoring therapy. However, there are only few studies using specific PET tracers in neuroblastoma patients. In other pediatric malignancies including germ cell tumors and hepatoblastoma PET and PET/CT may be helpful in individual cases, but the literature in these entities is limited so far. Although publications on the additional value of the combined PET/CT compared to both stand-alone modalities are still limited in pediatrics, it can already be anticipated that the combination of morphological and functional information obtained by integrated PET/CT will improve the accuracy of staging and will change patient management in a significant number of pediatric patients.