Composite malignancy of both Li Fraumeni syndrome (LFS) associated adrenocortical tumor (ACT) and neuroblastoma (NB) is extremely rare. We report a 10-month-old boy with a right sided abdominal mass that, after multiple resections, noted two separate tumors. The adrenal resection revealed characteristics notable for an ACT; the para-aortic and pelvic lymph nodes showed poorly differentiated NB. Additional analysis revealed a wild-type TP53 expression pattern and negative MYCN amplification, and germline testing revealed a heterozygous TP53 germline c.818G>A, pArg273His mutation, one only associated with separate tumors. This composite NB and ACT is thus the first reported in the literature.