en Ophthalmology Case Report and Review of Literature Case Report and Review of Literature <div style="text-align: justify;"><strong>Introduction</strong>: Melanocytoma is a rare benign stationary tumor that usually appears as a pigmented lesion on the&nbsp;optic&nbsp;disk. Optic Disc Melanocytoma (ODM) can compress the optic nerve or undergo necrosis, leading to ischemic axonal loss and visual field defect, similar to those caused by glaucoma. Also, ODM often displays a clinical diagnostic dilemma due to its similarities with melanoma. Some patients have undergone enucleation because of uncertainty between both pathologies. Progressive growth and malignant transformation can be documented by close monitoring of the patient&rsquo;s eyes. Fundus examination and ancillary imaging procedures such as fundus photo, autofluorescence, B-scan ultrasonography, fluorescein angiography, and spectral-domain optic coherence tomography are powerful tools for ODM diagnosis and management.&nbsp;<br> <strong>Case Presentation</strong>: A 19-year-old female presented with a decrease in vision in the left eye for about 3 months. Her visual acuity was 20/20 and 20/80 in her right and left eyes, respectively. Funduscopic examination of the left eye showed a well-defined deeply pigmented brownish-black, dome-shaped nodular mass covered the entire optic disc with the normal-appearing overlying vitreous, macula, and surrounding retina. Short-wave autofluorescence revealed hypo-autofluorescence on the pigmented mass lesion. The patient&rsquo;s condition did not change significantly over 2 years of follow-up. The diagnosis was made as ODM.<br> <strong>Conclusions</strong>: Melanocytomas grow very slowly over several years or remains stable, in contrast to malignant melanoma. Although ODM tends to have benign behavior, it may adversely affect visual function. Yearly fundus examination is necessary for monitoring growth and detecting malignant transformation. Visual loss can result from&nbsp;optic&nbsp;neuropathy or retinal vascular obstruction. In suspicious&nbsp;cases, close follow-up with serial fundus photographs is essential, even though the malignant transformation is exceptional.</div> Neoplasms, Eye, Fluorescein, Angiography, Tomography 61 66 http://jpr.mazums.ac.ir/browse.php?a_code=A-10-855-1&slc_lang=en&sid=1 Ali Ahmadzadeh Amiri 10031947532846006302 10031947532846006302 No Department of Pediatric, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran. Majid Reza Sheikhrezaee mr_sheikhrezaee@yahoo.com 10031947532846006303 10031947532846006303 No Clinical Research Development Unit of Bu-Ali Sina Hospital, Department of Ophthalmology, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran. Ahmad Ahmadzadeh Amiri ahmadzdh@yahoo.com 10031947532846006304 10031947532846006304 Yes Clinical Research Development Unit of Bu-Ali Sina Hospital, Department of Ophthalmology, Faculty of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.