Leptomeningeal metastasis (LM) is a severe complication of primary malignancy that has spread to the leptomeninges and cerebrospinal fluid (CSF). Here, we report a patient whose magnetic resonance imaging (MRI) showed a unique brainstem lesion suspicious of LM. A 72-year-old man presented with dizziness, gait instability, and cognitive decline, primarily object naming. He had a history of lung adenocarcinoma with epidermal growth factor receptor (EGFR) mutation. Brain MRI revealed a band-like lesion surrounding the ventral brainstem with T2 weighted-image/fluid attenuation inversion recovery (FLAIR) imaging and diffusion-weighted imaging (DWI) hyperintensity without gadolinium enhancement. No malignant cells were detected in the CSF. He underwent ventriculoperitoneal shunt two months after the onset, and his gait improved, but his cognitive function declined further. Recent reports suggest similar brainstem lesions as a unique LM pattern, which occurs almost exclusively in patients with lung adenocarcinoma with EGFR mutation. Therefore, if MRI shows this brainstem finding, repeated and appropriate CSF cytology is needed to detect tumor cells. Furthermore, if a patient with lung adenocarcinoma shows a cognitive decline, cerebral LM and auto-antibodies that mainly target neuronal surface antigens should be considered.