This review provides a comprehensive overview of the current molecular landscape of pineal region tumors, with a particular focus on genetic predisposition, somatic driver alterations, DNA methylation profiles, and transcriptional programs across pineocytoma, pineal parenchymal tumor of intermediate differentiation (PPTID), pineoblastoma, papillary tumor of the pineal region (PTPR), and desmoplastic myxoid tumor, <i>SMARCB1</i>-mutant. Here, SMARCB1 is linked to pineal parenchymal tumor of intermediate differentiation.