The peripheral-type benzodiazepine receptor (PBR) has been demonstrated to be critical for steroidogenesis in all steroid-producing tissues. Here, we review the identification and characterization of the PBR, the evidence pointing to its function as a cholesterol pore involved in transporting cholesterol from the cytoplasm of steroid-producing cells into the inner mitochondrial membrane where it is metabolized, and the known mechanisms regulating its function. We present data on the functions of the PBR in the adrenal gland, a classical steroidogenic tissue, and in the brain, which has only recently been proven to be steroidogenic. Finally, we discuss other potential roles for the PBR in pathological conditions, including cancer, neurodegeneration, and neurotoxicity, and a broader role for the PBR in mediating intracellular cholesterol transport/compartmentalization, which may or may not be linked to steroid biosynthesis.