The third case of leiomyosarcoma of the foreskin is reported. A review of the literature indicates that this lesion, which is similar to cutaneous leiomyosarcoma that is seen elsewhere in the body, has a low grade of malignancy. This finding is in contrast to leiomyosarcoma of the penis, which arises in deep structures and tends to metastasize. Circumcision appears to be the most effective treatment for leiomyosarcoma of the foreskin.