Lipid-lowering medications, particularly statins, have been a popular target for pharmacogenetic studies. A handful of genes have shown promise for predicting response to therapy from the perspective of lipid lowering, as well as myopathy. A number of genes have been implicated and have biological plausibility based on their involvement with the pharmacokinetics or pharmacodynamics of statins or other lipid-lowering medications. The level of confidence and replication of these findings varies, although several associations are likely true. Novel classes of lipid-lowering therapy have opened up new possibilities in the treatment of severe inherited forms of dyslipidemia, making the identification of such mutations an important pharmacogenetic predictor of failure of standard therapy, with potential response to novel therapy. Advances in next-generation sequencing technology bring the application of pharmacogenetics even closer to routine clinical practice.