Adenoid cystic carcinoma (AdCC) is an invasive malignancy 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, and often with gene fusions involving the MYB, MYBL1, and NFIB genes. The gene discussed is MYB; the disease is adrenocortical carcinoma, hereditary.