PLAGL1 (PLAG1 like zinc finger 1) is a zinc-finger transcription factor that functions as both a transcriptional activator and repressor, playing crucial roles in growth regulation, apoptosis, and development 1. The protein promotes cell cycle arrest and apoptosis through multiple pathways and acts as a growth suppressor 2. PLAGL1 is maternally imprinted and located on chromosome 6-25, a region frequently deleted in human cancers 1. The gene regulates imprinted genes, components of the Wnt/β-catenin pathway, and controls imprinting and differentiation processes 3. PLAGL1 has been identified as a regulator of disease-associated inflammatory states in fibroblasts 4. Clinically, PLAGL1 is associated with transient neonatal diabetes mellitus due to its imprinted status 1. The gene is involved in various pathological processes including metabolic and genetic disorders 2. Recent studies have identified PLAGL1 fusions and amplifications in pediatric CNS tumors, establishing new tumor classifications such as neuroepithelial tumors with PLAGL1 fusion and CNS embryonal tumors with PLAGL amplification 53. PLAGL1 hypomethylation has been associated with very preterm birth, suggesting its role in early development 6.