Gelatinous drop-like corneal dystrophy (GDLD) is a rare autosomal recessive corneal dystrophy that has been associated with mutations in the TACSTD2 (M1S1) gene, which is normally expressed in corneal epithelial cells. GDLD is characterized by progressive deposition of amyloid in the corneal stroma with rapid recurrence in grafts after penetrating keratoplasty. We report of case of a patient with GDLD treated bilaterally with staged limbal stem cell transplantation and penetrating keratoplasty that resulted in long-term control of his disease. This case demonstrates that staged allogenic limbal stem cell transplantation, before or after penetrating keratoplasty, can be used to restore vision long-term in GDLD patients.