Adenoid cystic carcinoma (AdCC) is an invasive carcinoma composed of epithelial and myoepithelial neoplastic cells arranged in tubular, cribriform, and solid patterns associated with an eosinophilic extracellular matrix and reduplicated basement membrane materials, often associated with gene fusions involving the MYB, MYBL1 and NFIB genes. The gene discussed is NFIB; the disease is adrenocortical carcinoma, hereditary.