Pharmacogenetics is being applied to develop individual specific therapies considering different ethnic groups and mixed populations. The Venezuelan population is very heterogeneous as a result of the admixture process that occurred between Native Americans, Europeans, and Africans through five centuries. This review provides a summary of the literature concerning gene variants within drug-metabolizing enzymes, drug targets, and drug receptors (CYP2C19, CYP2D6, GSTM1, GSTT1, GSTP1, NAT2, MTHFR, LEP, LEPR, LTC4S, and ADRβ2 genes) evaluated in the Venezuelan population. In particular, most of the studies were conducted with relatively low numbers of individuals. Some of these studies included analyses of genetic polymorphisms in native groups living in this country. Although the recent studies represent a hopeful progress toward the inclusion of the Venezuelan population among those who will benefit from the implementation of pharmacogenetic principles and tools in drug therapy, there are not yet sufficient data concerning allelic frequencies of genomic biomarkers related to drug response for their implementation in clinical practice. Therefore, there is a critical need for more research in pharmacogenetics in Venezuela to increase data availability.