Introduction: Cystic brain lesions represented a rare finding in cancer patients, even more uncommon in
rare disease as neuroendocrine neoplasms. Patient Presentation: We presented a case of a patient with
neurologic symptoms as first and only presentation of a neuroendocrine tumor of the lung. Management
and Outcome: First of all, the patient underwent neuroradiologic assessment with the finding of cystic
brain lesions. A chest and abdomen CT scan detected a pulmonary lesion; a biopsy of this mass revealed an
atypical carcinoid of the lung. Considering the absence of other symptoms, in order to clarify the nature of
the cystic brain lesions, a right frontal craniotomy was performed, and a cyst was completely excised.
Pathology confirmed the metastatic nature of the brain lesions. Unfortunately, the conditions of the patient
quickly worsened, and he passed away before the start of the treatment. Conclusion: Due to the low
awareness about neuroendocrine tumors and their clinical and radiological presentations, multidisciplinary
approach can represent the cornerstone to provide an accurate and timely diagnosis.