Case 2. A 12-year-old boy (family 2, II:1) was referred for elevated IOP. He had no history of trauma or surgery. On examination, his best-corrected visual acuity was 20/20 OU with −3.00 D OD and −3.75 D OS. He had a Krukenberg spindle in both eyes. Corneal pachymetry was 470 μm OD and 487 μm OS. Radial transillumination defects were noted in both eyes. His IOP was 26 mm Hg OD and 31 mm Hg OS. The angles were wide open with dense trabecular pigmentation. After pupillary dilatation, moderate pigment dispersion was noted in the anterior chambers but the IOP remained the same. The lenses were clear with Zentmayer rings. The cup-disc ratios were 0.60 OD with a healthy neuroretinal rim and 0.65 OS with early thinning of the superotemporal rim. Humphrey visual fields (24-2) and multifocal visual evoked potential testing results were within normal limits. Optical coherence tomography showed possible focal thinning of the superotemporal quadrant in the left eye, while Heidelberg retinal topography showed borderline thinning of the rim superiorly in both eyes and results from scanning laser polarimetry with variable corneal compensation were within normal limits in both eyes. Argon laser peripheral iridotomy was performed in 1 eye, and after results were judged to be beneficial by a reduction in the IOP, argon laser peripheral iridotomy was performed in the fellow eye 1 year later (Figure 4). Both of the boy's parents had PDS with normal IOP, with greater pigmentation in the father than in the mother (Figure 1, family 2).