Objective:The objectives of this study were to examine common patterns in the lives and artwork of five artistic savants previously described and to report on the clinical, neuropsychological, and neuroimaging findings from one newly diagnosed artistic savant.Background:The artistic savant syndrome has been recognized for centuries, although its neuroanatomic basis remains a mystery.Methods:The cardinal features, strengths, and weaknesses of the work of these six savants were analyzed and compared with those of children with autism in whom artistic talent was absent. An anatomic substrate for these behaviors was considered in the context of newly emerging theories related to paradoxical functional facilitation, visual thinking, and multiple intelligences.Results:The artists had features of “pervasive developmental disorder,” including impairment in social interaction and communication as well as restricted repetitive and stereotyped patterns of behavior, interest, and activities. All six demonstrated a strong preference for a single art medium and showed a restricted variation in artistic themes. None understood art theory. Some autistic features contributed to their success, including attention to visual detail, a tendency toward ritualistic compulsive repetition, the ability to focus on one topic at the expense of other interests, and intact memory and visuospatial skills.Conclusions:The artistic savant syndrome remains rare and mysterious in origin. Savants exhibit extraordinary visual talents along with profound linguistic and social impairment. The intense focus on and ability to remember visual detail contributes to the artistic product of the savant. The anatomic substrate for the savant syndrome may involve loss of function in the left temporal lobe with enhanced function of the posterior neocortex.(NNBN 2000;13:29‐38)