Gelatinous drop-like corneal dystrophy is a rare disease, described for the first time by Nakaizumi (Japan) in 1914. We report 5 cases of this primary corneal amyloidosis. Three of our patients are brothers, the two other cases are isolated cases. Photophobia and progressive loss of vision are the first presenting signs. Corneal lesions are typically bilateral white nodular deposits beneath the epithelium. Patients underwent repeated keratoplasties with a mean interval of 5 years because of the recurrence of the disease on the corneal graft. Diagnosis of corneal amyloidosis was confirmed in all cases by histology. We compared our results with the data reported in the literature.