Purpose: To describe the macular holes in patients with idiopathic parafoveal telangiectasis (IPT) and, to propose a pathophysiologic explanation for their formation. Methods: Four eyes of two patients with IPT were evaluated with biomicroscopy and optical coherence tomography (OCT). Results: One patient had a nearly full-thickness hole with preservation of only the internal limiting membrane (ILM), but had a 20/60 visual acuity. The other patient had a large full-thickness macular hole, but retained 20/40 visual acuity. Each patient had. a fellow eye showing prominent central inner foveal cavitation under a very thin ILM, which was devoid of associated tissue. Conclusions: This report describes the findings of two patients with IPT who developed pronounced central foveal structural abnormalities. The induced anatomic changes noted in our patients suggest that there is a loss of the structural aspects afforded by Muller cells, particularly the Muller cell cone, in the central macula in patients with IPT. The preservation of good visual acuity in our patients implies that the holes were the result of lateral separation of the photoreceptors within the fovea and that there could not have been profound atrophy of the photoreceptors.