BackgroundPulmonary adenoid cystic carcinoma (PACC) is an exceptionally rare salivary gland-type malignant neoplasm. Because of its clinical manifestations, imaging features are not different from other types of non-small cell lung cancer, which is a diagnostic challenge for most doctors.ConclusionsA review of the literature shows that high amounts of immunohistochemical (IHC) markers, such as CK7, CD117, P63, SMA, CK5/6, and S-100 are helpful for PACC diagnosis. Surgical resection is the main treatment of PACC, but treatment options for advanced PACC patients are limited and the research of molecular targeted drugs is ongoing in advanced cases not eligible for surgery. Currently, research on PACC targeted therapy mainly focuses on the exploration of v-myb avian myeloblastosis virus oncogene homolog (MYB) and its downstream target genes. In addition, median tumor mutation burden and PD-1/PD-L1 were lower in PACC, which may indicate poor efficacy of immunotherapy in PACC patients. This review focuses on the pathologic features, molecular characteristics, diagnosis, treatment and prognosis of PACC to establish a comprehensive understanding of PACC.