This review aims to explore the molecular landscape of ACC arising in the lacrimal gland and analyse established genetic mutations described in the literature that are key drivers of disease pathology, including fusion translocations, such as the MYB–NFIB fusion, Notch signalling, DNA damage response genes, and epigenetic modifications, and chromatin remodelling genes, such as SMARCA2, CREBBP and KDM6A [12,34]. This evidence concerns the gene SMARCA2 and adrenal cortex carcinoma.