According to [PERSON], to correctly diagnose an iris melanoma, the lesion should replace the stroma of the iris, should be larger than 3 mm in diameter and 1 mm in thickness and should present at least three of the following five features: photographic evidence of growth, secondary cataract, glaucoma, conspicuous vascularity and iris ectropion [22]. [PERSON] and [PERSON] believe that ectropion uvea, vascularity, pupil distortion, involvement of the angle and glaucoma could be evident in other types of lesions (nevus or melanoma) [23]. They propose only the involvement of the ciliary body as the most indicative aspect of malignancy, while [PERSON] is of the opinion that documented growth is the most important sign to diagnose iris melanoma [22]. After the evaluation of 1611 eyes, [PERSON] et al. constructed an ABCDEF guide to remember the risk factors that are predictive of iris nevus growth to melanoma, where A is age (young), B is blood (past episodes of hyphema), C is the clock hour (a tumor location from 4 o'clock to 9 o'clock), D is a diffuse configuration, E is ectropion and F is a feathery margin. These key clinical features help to identify iris melanoma at a time in which therapy could be life-saving [22]. The main local clinical complications of iris melanomas are tumor vascularization (in terms of sentinel episcleral vessel and posterior iris feeder vessel), ectropion uvea, pupillary distortion, pigment dispersion, sector cataract, chronic uveitis [19], hyphema and glaucoma with irreversible optic nerve damage [23]. The most common mechanism of elevated intraocular pressure (IOP) in patients with iris tumor is the invasion of the trabecular meshwork with outflow reduction, whereas the anterior displacement of the iris and lens and iris neovascularization, leading to angle closure, are the main mechanisms in choroidal melanoma [17]. [PERSON] et al. demonstrated that an elevated IOP and iris root invasion are risks that are almost exclusively related to iris melanoma; in fact, increased IOP is encountered in approximately 7-30% of patients with iris melanoma and only 2% of those with choroidal melanoma [17]. Moreover, [PERSON] et al. analyzed the clinical factors that are statistically associated with glaucoma: the seeding of melanoma into the anterior chamber angle, large tumor size, the tumor epicenter being at the iris root, a flat shape and a visual acuity less than 20/20 [17]. In general, the greater the clock hour involvement with angle seeds, the greater the IOP. In addition, in an eye with iris melanoma and glaucoma, the likelihood of metastasis increases considerably [17]. For a precise patient diagnosis, the following lesions in the iris need to be differentiated: congenital heterochromatin, congenital ectropion iridis, [PERSON] syndrome (iridocorneal endothelial syndrome, which usually affects one eye of young to middle-aged women), siderosis, hemosiderosis, pigmentary glaucoma, melanocytanalytic glaucoma [21], Koeppe or Busacca nodules characteristic of sarcoidosis, Lisch nodules in neurofibromatosisis, iris nevi, iris cysts, iris metastases, leiomyoma, juvenile xanthogranuloma and ciliary body melanoma with anterior extension [7].