Objective: To test the hypothesis that the macular pigment maybe a marker of foveal cone function and consequently the structural integrity of foveal cones and further of evaluate the Correlation of the phenotype and genotype on Macular Stargardt dystrophy. Methods: Sixteen patients (32 eyes)diagnosed to have Stargardt dystrophy by clinical were studied with a scanning laser ophthalmoscopy (SLO) comparing argon laser blue and infra-red images for the presence or absence of macular pigment (MP)in the fovea. Fifteen of the STGD patients were screened for mutations in the ABCR gene. An C computer based program was used to evaluate the density of MP. Eyes were graded into three categories: those without foveal macular pigment, those with partial pigment and those with normal amounts of macular pigment. These categories were compared with visual acuity determined by the Snellen chart. And the relationship of STGD genotype and phenotype was evaluated. Results: All patients with a visual acuity of 20/200 or worse had no macular pigment in the fovea. All patients with visual acuity of 20/40 or better had a normal of macular pigment in the fovea. Patients with partial macular pigment had intermediary acuity values except for two eyes, one with 20/20 and another with 20/200 acuity. There was a tendency for patients with mutations near the 5’end of the ABCR gene to have a more severe phenotype than with defects near the 3’end . Conclusions: Foveal macular pigment is closely related to foveal cone acuity and therefore may be a marker for the presence of foveal cones, Gene defects towards the 5’end of the ABCR gene are more severe than defects towards the 3’end . Infrared light is a sensitive indicator of early Stargardt’s disease.