A 22-year-old white man presented with photopsia of 3-year duration despite a corrected distance visual acuity of 20/20 in both eyes. Ophthalmic examination revealed multiple irregularly shaped white cortical opacifications of the lens in both eyes but no other significant ocular findings. One year after the photopsia onset, the patient experienced an episode of seizures with generalized tonic-clonic movements. Neurological follow-up examination demonstrated extensive brain calcification in the basal ganglia and frontal lobes, leading to a diagnosis of pseudohypoparathyroidism. Pseudohypoparathyroidism is a rare condition, and there are few reports of ocular findings with this underlying pathology. Our findings clearly demonstrate that even relatively unremarkable ocular signs can lead to diagnosis of severe systemic diseases. Therefore, the cataract surgeon should be aware of these conditions before removing the lens.