In this review, the brain substrate underlying outstanding artistic ability was discussed from the viewpoint of "neuroaesthetics" and savant syndrome. Beauty is a multifaceted concept and is related to other multiple cognitive or affective functions, for which the medial orbitofrontal cortex is the hub and core region of the brain. The savant syndrome is known for outstanding and talented abilities including art despite otherwise severe brain dysfunction. The savant syndrome is usually classified into the essential savant observed in autism spectrum disorders and acquired savant often observed in dementia/degenerative diseases or focal brain damage. The paradoxical functional facilitation hypothesis may account for the savant syndrome, in which focal brain dysfunction may paradoxically enhance/facilitate functions in the other areas of the brain. This implication is important and useful in understanding the recovery/treatment of neuropsychiatric conditions.