With an objective to review the clinical presentation, diagnosis, and management of immune checkpoint inhibitor (ICPi)-associated hypophysitis and pituitary dysfunction, we conducted a systematic review of 84 studies (2005-2025) involving 7,259 patients evaluated pituitary immune-related adverse events (irAEs) linked to CTLA-4 inhibitors, programmed cell death protein 1 (PD-1)/programmed cell death-ligand 1 (PD-L1) inhibitors, and combination therapies. The gene discussed is CD274; the disease is hypophysitis.