The limbic system is predominantly involved in memory and emotional output. Its 2 principle components are the hippocampus (involved in memory as part of the Papez circuit) and the amygdala (involved in emotional responses, memories and drives). The principle clinical manifestations of limbic disease are epilepsy, confusional states, and cognitive impairment. The connections of the limbic system are widespread and are now becoming visible on diffusion tensor imaging. Many different diseases may affect the limbic system. An appreciation of its functional anatomy along with its white matter tract connections improves assessment of infiltrative disease in particular. Small lesions in the Papez circuit may have devastating neuropsychological consequences. An active search strategy based on the knowledge presented in this paper will increase the likelihood of making an accurate diagnosis for patients affected by these conditions.