A 50-year-old woman presented with conjunctival melanosis, scleral pigmentation, and Lisch nodules in her left eye. Intraocular pressure was 24 mmHg in the right eye and 14 mmHg in the left eye. She had open angles on gonioscopy. Fundus examination showed a cup-to-disc ratio of 0.7 in the right eye, with an inferior notch and a splinter hemorrhage, and 0.6 in the left eye, with a deep cup with sloping rims. Humphrey visual fields showed an evolving superior arcuate scotoma in her right eye; the left eye was normal. Systemic examination showed axillary freckling. The patient had a family history of neurofibromatosis type 1 (NF-1), her father having been diagnosed with the condition. She had hyperpigmentation of the skin over the forehead and periocular skin on the left side. These unique ocular and systemic features were suggestive of two phakomatoses, NF-1 and nevus of Ota, in one eye, and primary open-angle glaucoma (POAG) in the other eye. that is, three pathologies present together in the same individual, which is an extremely rare occurrence.