Using a population-level cohort analysis, our study demonstrates that, although rare, de novo autoimmune cutaneous connective tissue diseases (AiCTDs) in the setting of immune checkpoint inhibitors (ICIs) are not associated with a greater risk of mortality and overall approach a statistically significant decrease in mortality when compared with patients treated with ICIs who do not experience cutaneous immune-related adverse events. These findings are significant and highly relevant to dermatologists and oncologists caring for ICI recipients as it adds to the limited information on development of cutaneous AiCTD following ICI administration, for which enhanced understanding is critical to improving the care for this challenging patient population.