Peyronie's disease is an idiopathic, localized connective tissue disorder of the penis that involves the tunica albuginea of the corpus cavernosum and the adjacent areolar space. The tunica albuginea plays an important role in the mechanism of erection. Peyronie's disease is characterized by local changes in the collagen and elastic fiber composition of the tunica albuginea. The formation of fibrotic plaques alters penile anatomy and can cause different degrees of bending, narrowing, or shortening of the penis. Moreover, a significant number of men with Peyronie's disease develop erectile dysfunction. Penile blood flow studies in many patients with Peyronie's disease suggest a strong association with veno-occlusive dysfunction. Although long recognized as an important clinical entity of the male genitalia, the etiology of this disease has remained poorly understood. The following review focuses on recent research on the pathophysiology of Peyronie's disease.