A 57-year-old woman diagnosed with angioid streaks, retinal flecks and chorio-retinal Stargardt-like atrophy due to PXE (homozygous nucleotide substitution in exon 9 of the ABCC6 gene [C1132T] introducing a stop codon at position 378 [Q378X]) was referred to our department for sudden decreased vision in her right eye (RE). Here, ABCC6 is linked to Pseudoxanthoma elasticum.