We describe the light microscopic, immunohistochemical, and electron microscopic findings in a sporadic case of congenital microcoria in a 72-year-old man with senile cataract. We demonstrated a lack of myofilaments and desmin in the stromal cytoplasmic processes of the anterior pigmented cells of the iris, although other features of muscle differentiation were present in these few surviving cell processes that normally form the pupil dilator muscle. Degenerative changes in anterior pigment cells and iris stromal atrophy were thought to be late secondary features of microcoria. The findings suggest that congenital microcoria results from a defect of intermediate filaments in the terminal fetal stages of differentiation of the anterior pigmented epithelial cell of the iris, with absence of myofilaments and consequent failure of development of a functional dilator pupil muscle.