Here, we utilized a broad genome-wide DNA methylation cohort, combined with copy number profiling, targeted next-generation DNA sequencing, and RNA sequencing, to identify a rare CNS tumor type characterized by amplification and overexpression of either PLAGL1 (located at chromosome 6q24.2) or PLAGL2 (located at chromosome 20q11.21). This evidence concerns the gene PLAGL1 and central nervous system neoplasm.