Nine patients with congenital retinoschisis were examined. Two patients showed only the stellate c cystoid macular schisis without peripheral changes. Most of the affected eyes demonstrated a vision between 6/15 and 6/21. Fluorescein angiography did not reveal any macular abnormalities, even though changes were ophthalmoscopically visible. Patients in their twenties developed nonspecific macular degeneration, but did not have acceleration of visual deterioration.