Introduction
Li Fraumeni syndrome (LFS) is characterized by TP53 mutations and increases susceptibility to tumors, such as sarcoma, breast cancer, brain tumors, and adrenocortical tumor (ACT)1. Neuroblastoma (NB) is the most common malignancy of the adrenal medulla in infants and is notably associated with the MYCN gene amplification. Composite malignancy in adrenal glands or other organs is extremely rare and usually seen in adults; only a weak association between LFS and NB has been reported.2,3 Simultaneous ACT and NB has only been reported in six other cases, all with varying TP53 mutations.