Programmed cell death plays a critical role in maintaining organism growth and development, homeostasis and physiological functions of organs. Ferroptosis suppressor protein 1 (FSP1) is capable of regulating not only apoptosis but also ferroptosis, mainly dependent on its intracellular distribution, yet associated mechanisms still unclear. This review summarizes the role and regulatory mechanism of FSP1 in two types of programmed cell death, by which new strategies can be provided for the diagnosis and treatment of tumors, neurodegenerative diseases and other diseases related to programmed cell death via precisely regulating the expression and distribution of FSP1 in cells.