As has been demonstrated, Gilles de la Tourette Syndrome (GTS) occurs in at least 1% of the population worldwide. However, earlier studies suggested a lower prevalence. In addition, the prevalence figures for different studies very between 0.4% and 3.8%. Moreover, the prevalence appears to vary in some parts of the world and races, with a lower rate in Afro-Americans and sub-Saharan black Africans. In this the second part of the review, possible reasons for the differences in prevalence and epidemiology are discussed. Tentative explanations for differing prevalence figures in GTS include problems with the diagnosis of GTS, the multidimensional nature of tics, as well as other tic factors including the waxing and waning of symptoms and the suppressibility of symptoms. Other factors inherent to GTS include the fact that there is no diagnostic test and indeed no definitive diagnosis other than clinical, the fact that psychosocial stresses can lead to increased tic severity, and that comorbid disorders may mask tics. The varying methods of study employed can also effect prevalence. There may be some regional differences in GTS as well, which may be due to a lack of awareness of GTS, or it may be a true reflection of low prevalence as in some populations GTS does appear rare. With regard to the sub-Saharan Africa data and possibly the African American data, matters are much more complex than meets the eye. The following reasons are all possible for the apparent rarity in these populations and include (i) other medical priorities and less propensity to seek health care, (ii) lack of awareness of GTS, (iii) chance, (iv) ethnic and epigenetic differences and reasons, (v) genetic and allelic differences in different races, and (vi) an admixture of races. The aetiology of GTS is also complex, with influences from complex genetic mechanisms, pre- and perinatal difficulties and, in a subgroup, some infections, possibly by epigenetic mechanisms. These may well affect phenotype and, thus, prevalence. There have even been suggestions that people with GTS are increasing. Recent data suggests that GTS is not a unitary condition and that there may well be different types of GTS. The prevalence of GTS in these individual subtypes is unknown. It is suggested that a new nomenclature be adopted for GTS in future, pending further genetic and phenomenological studies. To what extent the aetiology affects the phenotype and, thus, the prevalence is still unclear.