Frequent mutations in multiple endocrine neoplasia 1 (MEN1; 44%), death domain-associated protein (DAXX)/chromatin remodeler (ATRX; 43%), mTOR (15%) pathway genes, and Von Hippel Lindau (VHL) alongside several other hereditary disorders are observed in PNET [8]. The gene discussed is DAXX; the disease is primitive neuroectodermal tumor.