A 50-year-old male patient presented to the neuro-ophthalmology clinic with chief complaints of gradual decrease in vision in both eyes, more in the left eye, for 6 years. On general examination, the patient had a hemiplegic gait. His presenting acuity was 20/50 in the right eye and 20/320 in the left eye, not improving further. He had dense posterior subcapsular cataracts in both eyes, and fundus examination revealed pale discs. Humphrey visual field tests 30-2 revealed a vertical nasal midline defect in the right eye and grossly depressed fields in the left eye. Keeping in mind the above findings, the authors requested for a magnetic resonance imaging (MRI) of the brain. The brain MRI shows a large infarct in the right parieto-occipital lobe and a small circumscribed lesion in the left cerebellum. The radiologist opined that it could possibly be a gangliocytoma of the cerebellum, and a possible diagnosis of Lhermitte-Duclos syndrome was made.