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There are reports on the successful management of mild to moderate keratoconus in patients with Down's syndrome. However, when Down's syndrome is coexistent with Dementia, poor general health, and very advanced keratoconus in only one eye, then clinicians may face challenges at all stages from diagnosis, treatment, and follow-up. Our case is one such example of a combination of Down's syndrome, Dementia, poor general health, and very advanced keratoconus with a white cataract in the only functioning eye. This report highlights the dilemmas which were encountered and learning points on selection of the safest, yet effective management options in such patients.   How to cite this article  Ali H, Nanavaty MA. Clinical Dilemmas in the Management of a Monocular Patient with Down's Syndrome, Dementia, Advanced Keratoconus, and a Mature Cataract. Int J Kerat Ect Cor Dis 2016;5(2):81-84.