The authors report a case of a 2-year-old male with chronic granulomatous disease (CGD). On examination, the patient had chorioretinal lesions, peripheral avascular retina, and unilateral glaucoma. CGD is a primary immunodeficiency that leaves the host susceptible to infections and granuloma formation. Chorioretinal lesions are a well-documented finding of CGD. The advent of widefield angiography allowed the authors to detect peripheral retinal nonperfusion in this case. Glaucoma, which by itself has been associated with peripheral avascular retina, has not been previously reported in a case of CGD.