The novelty of the present report relies on the association between the MEN1 pathogenic variant (NM_130799.2):c.758delC (p.Ser253Cysfs*28) in exon 4 and a clinical phenotype characterized by pHPT, non-functioning pituitary microadenoma, left adrenal adenoma and multifocal pancreatic NETs. The gene discussed is MEN1; the disease is adrenal cortex adenoma.