This 43 year old woman first noticed some reduction in visual acuity in both eyes at age 14. Her visual acuity had been previously normal.

The clinical features favoring the diagnosis of ELOVL4-associated Stargardt-like dominant macular dystrophy include: loss of acuity in the second decade; horizontally oval geographic atrophy centered on fixation and surrounded by yellow pisciform flecks; and, a family history compatible with autosomal dominant disease.