This concise review provides an overview of the biological role of DICER1, the most important intracranial manifestations of DICER1 syndrome, and reviews somatic and germline distributions in four prototypic DICER1-associated tumors (intracranial sarcoma, pineoblastoma, ETMR, and pleuropulmonary blastoma). Here, DICER1 is linked to pineoblastoma.