Fahr’s disease is a rare neurodegenerative disorder characterized by symmetrical calcifications of the basal ganglia. Fahr’s disease is categorized as a neurodegenerative condition due to the pathophysiological effects of calcification. The condition can be associated with a range of neuropsychiatric, motor, and cognitive symptoms. We report a 38-year-old man with mania with psychotic features. Neurological examination showed a bilateral tremor in the hands. Imaging revealed calcifications in the basal ganglia, consistent with Fahr’s disease. He was successfully treated with olanzapine and lithium, but developed extrapyramidal symptoms, which responded well to procyclidine. The risk of extrapyramidal symptoms and neuroleptic malignant syndrome is increased in Fahr’s disease, necessitating cautious use of antipsychotic medications. The complex symptomatology and incomplete penetrance of Fahr’s disease may contribute to underdiagnosis. This case highlights the value of brain imaging in cases of severe atypical psychiatric symptoms.