The centrosome, located in the perinuclear region of eukaryotic cells, serves as the major microtubule organizing center. It has been documented that centrosomes appear to be involved in many physiological processes, whose homeostasis prevents the onset of multiple diseases. Apart from their roles in cell division, motility, and polarity, centrosomes are also involved in the host immune response and a diverse range of inflammatory diseases. In this article, we will review the main functions and signaling pathways involving the centrosomes in immune cells and associated diseases, providing a reference for further investigation into their immunological effects and regulatory mechanisms.