Abstract. Alveolar adenoma is an extremely rare benign tumor of the lung derived from type II pneumocytes. To date, only a limited number of alveolar adenoma cases have been reported in the literature. These tumors are usually discovered incidentally on radiographic images. However, other tumor types must be excluded when the characteristics of alveolar adenoma are not typical on the radiographic images. Therefore, diagnosis of alveolar adenomas is based on the combination of gross structural analysis and immunohistochemistry. The present study described a case of alveolar adenoma in a 47-year-old female patient. A chest X-ray revealed a pulmonary lesion in the right lower lobe and, subsequently, the patient underwent a right lower lobectomy. Pathological examination of the surgically resected tissue confirmed the initial diagnosis of alveolar adenoma. In addition, intracranial vascular malformations were detected on a computed tomography scan, but the patient did not experience any notable symptoms. Considering the benign nature of the malformations, as determined by digital subtraction angiography, the patient selected to undergo follow-up with observation only. The postoperative course was uneventful and no recurrence of pulmonary lesions was noted after >4 years, while the condition of the intracranial lesion was stable. In conclusion, the current study described a unique case of adenoma alveolar coexisting with intracranial vascular malformations. A literature review of previously-reported alveolar adenoma cases was also conducted.