Schnyder corneal dystrophy (SCD) is a rare, autosomal dominant, anterior stromal dystrophy caused by a mutation in the UBIAD1 gene on chromosome 1p. It is characterized by the accumulation of crystals in corneal stroma containing cholesterol and neutral fat, which stains red with oil red O. Clinically, the crystals appear yellow and white and can be associated with corneal haze and corneal arcus. The gene discussed is UBIAD1; the disease is Schnyder corneal dystrophy.