This chapter provides a visual synopsis of the complex interplay between systemic diseases and corneal pathology. Vivid photographs illustrate the corneal manifestations of lysosomal storage diseases, highlighting the classic corneal clouding and unique storage material deposits. The chapter also depicts the corneal involvement in Goldenhar syndrome. An interesting image of congenital rubella syndrome leading to sclerocornea is provided. This is followed by corneal involvement in Hansen’s disease and PUK with perforation secondary to Wegner’s granulomatosis. Lastly, there is a pictorial depiction of nutritional keratopathy secondary to protein energy malnutrition, adherent leukoma in Peter’s anomaly, and acute hydrops in Down syndrome. This chapter serves as a crucial guide for clinicians in recognizing the often-subtle corneal signs of systemic conditions.