The killer-cell immunoglobulin-like receptors (KIR) form a diverse family of receptors that control the functions of natural killer cells. Sequencing of KIR from primates has revealed the unexpected extent to which this gene family has diversified mostly likely in response to pathogens and to pathogen-mediated selection of their MHC class I ligands. Human KIR diversity is now a burgeoning area for disease association studies. This review examines the evolution of KIR from a primate-centric view in order to rationalize our current knowledge of the diversity of human KIR.