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Background: we report a rare case of late-presentation keratoconus with acute hydrops in a woman in her 60s with oculocutaneous albinism (OCA). Keratoconus is a progressive non-inflammatory corneal ectatic disorder that usually presents in adolescence. 1 OCA is a hereditary condition characterised by hypopigmentation of skin and ocular abnormalities.2 The coexistence of corneal ectatic disorders and OCA is rare, with only three cases documented so far.3–5