The origin of amyloid in gelatinous drop-like corneal dystrophy, one of the primary corneal amyloidoses, was studied by light and electron microscopy. Milky-white granules over the corneal surface are the first clinical findings. In the initial stage, the amyloid substance exists between the basal cell of the corneal epithelium and the basal lamina of the cell. The basal lamina, Bowman's membrane and the stroma remained intact. Numerous filaments, about 7nm in width, were seen in the normal basal cells. In addition, many fibrils, about 10nm in width, were observed in the damaged basal cells. With progressing of the disease, the amyloid increased in amount and Bowman's membrane disappeared. Our results suggest that the basal cell of the corneal epithelium may produce and secreted the amyloid substance and gelatinous drop-like corneal dystrophy occurred.