Multiple primary malignancies are defined as two or three malignant neoplasms arising in different organ systems. Several cases of multiple primary malignancies are emerging in recent years due to the advancement in medical therapy and diagnostics. Multiple primary malignancies are not uncommon occurring at 0.7-16% of cancer patients, however, reported cases of multiple primary sarcomas are sparse. Presented in this report is a pediatric patient diagnosed with primary metachronous cerebral rhabdomyosarcoma after being treated for primitive neuroectodermal tumor/Ewing’s sarcoma of the oral cavity. Despite limited cases addressing multiple primary sarcomas, this entity must not be overlooked as it is associated with a meager outcome compared to an index case of sarcoma alone.