We examined 194 patients with oculodermal melanocytosis. Dermal involvement alone was present in 67 (34.5%) patients, while 12 (6.2%) had only ocular involvement. The remaining 115 (59.3%) patients had both ocular and dermal pigmentation. Dermal hyperpigmentation in the combined distribution of the ophthalmic and maxillary divisions of the trigeminal nerve and hyperpigmentation of the nasal or buccal mucosa were closely associated with ocular involvement. Ocular hyperpigmentation most commonly involved the episclera. Associated ocular findings included elevated intraocular pressure with or without glaucoma (10.3%), uveitis (2.6%), cataract (1%), asymmetric cupping of the optic nerve head unassociated with glaucoma (9.8%), and orbital melanoma (0.5%). The most serious complication of oculodermal melanocytosis is malignant transformation, while glaucoma appears to be the more common one. Patients with oculodermal melanocytosis and ocular hyperpigmentation should be followed up at regular intervals for the development of either of these complications.