Objective To test the hypothesis that the macular pigment may be a marker of foveal cone function and consequently the structural integrity of foveal cones. Methods Sixteen patients (32 eyes) diagnosed to have Stargardt dystrophy and three patients with full thickness macular holes by clinical criteria 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. 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. Results Thirteen eyes with a visual acuity of 20/200 or worse had no macular pigment in the fovea. Eleven eyes with visual acuity of 20/40 or better had a normal amount of macular pigment in the fovea and 1 eye had partial macular pigment. Eleven eyes with partial macular pigment had intermediary acuity value. Conclusions Foveal macular pigment is closely related to foveal cone acuity and therefore may be a marker for the presence of foveal cones. Infrared light is a sensitive indicator of early macular diseases