Background/Objectives: Acinic cell carcinoma (ACC) is a rare lung neoplasm that can affect both children and adults as a parenchymal or endobronchial mass. It is histologically similar to this kind of tumor described in salivary glands, but with a different immunophenotype. In general, it poses a reduced degree of malignancy, with indolent growth and a favorable prognosis, with exceptionally rare cases associated with recurring disease or lymph node metastases. Methods: When clinicians are facing puzzling symptomatology in their patients, the main role of the multidisciplinary team in their review of oncological cases is to recommend imagistic-guided biopsies. Tissues samples were routinely processed, stained with hematoxylin-eosin (HE) and periodic acid–Schiff (PAS), and submitted to complementary immunohistochemistry tests. Results: Histopathological reports were consistent for lung ACC with regional lymph node involvement and remote metastases. Oncological therapies followed. Conclusions: Postponements of the presentation to the doctor at the onset of symptoms, as well as a lack of periodic health control for people insured by national health insurance companies, often lead to medical, human and financial complications that are difficult to manage. The pathologist involved in the discussion of oncological cases brings his expertise in solving cases, certifying the evolution of tumors considered less aggressive, such as in the case of lung ACCs.