Background <p>Gelatinous drop-like dystrophy is a rare autosomal recessive disorder caused by pathogenic variants in the <i>TACSTD2</i> gene and characterized by subepithelial amyloid deposits in the cornea. Alterations in <i>FOXC2</i> are seen in lymphedema-distichiasis syndrome, in which patients classically exhibit lymphedema of the extremities and a double row of eyelashes. This report links alterations in the <i>FOXC2</i> gene to a corneal phenotype that is histologically identical to that seen in gelatinous drop-like dystrophy.</p> Case presentation <p>A 14-year-old male with congenital distichiasis presented with chronic photosensitivity and progressively worsening vision over several years. On examination, gelatinous-appearing lesions were observed on the inferior third of each cornea with neovascularization. Genetic testing identified a likely pathogenic alteration in <i>FOXC2</i>, a gene linked to lymphedema-distichiasis syndrome. No genetic alterations of corneal dystrophy were identified. The patient underwent excisions of the corneal lesions with simple limbal epithelial transplant, amniotic membrane transplant and temporary central tarsorrhaphy. The pathologic features of the corneal lesions resembled gelatinous drop-like dystrophy. One side was free of inflammation, while the other side showed a granulomatous response to the amyloid, which is unusual in this site. Potential pathogenic and clearance mechanisms are discussed. There has been no recurrence of the corneal lesions in approximately 3 years of follow-up.</p> Conclusions <p>This case presents a new link between <i>FOXC2</i> alterations and a histological phenotype that resembles gelatinous drop-like dystrophy (GDLD-like), emphasizing the importance of clinical, pathologic, and molecular correlation. Granulomatous responses to amyloid are unusual in this immune privileged site.</p>