Background: Nephropathic cystinosis is a rare genetic disease characterized by accumulation of the amino acid cystine in lysosomes throughout the body. Ocular manifestations, particularly corneal cystine crystal formation, remain a major complication of nephropathic cystinosis.Methods: We studied anterior and posterior segment ocular manifestations in twenty patients with confirmed nephropathic cystinosis above two years of age. In addition to oral cysteamine, topical cysteamine eye drops were provided and Corneal Cystine Crystal Scoring (CCCS) was followed up every 3 months over a period of 12 months.Results: All patients had corneal cystine crystals associated with variable degree of photophobia, 13 of them had also conjunctival crystals. Funduscopy was normal in 17 patients and posterior segment abnormalities were detected in only 3 patients; optic nerve disc elevation in two patients and mottling pigmentary changes in the third patient. After 12 months of cysteamine eye drops, CCCS didn't increase from the initial value and was associated with decrease of 1.0 in 1 patient and 0.5 in 12 patients. Compliance was generally inadequate due the need for high frequency of administration and multi-drug regimen.Conclusion: Anterior segment ocular manifestations of nephropathic cystinosis are more frequent than posterior segment involvement, being uniformly present in almost all patients beyond infancy. Topical cysteamine therapy is effective in depleting cystine crystals from the avascular cornea. Nevertheless, there is a pressing need for long-acting cysteamine eye drops to enhance patient compliance and improve outcome.