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An unusually large onychomatricoma. Onychomatricoma is a rare benign tumour of the nail matrix first described in 1992, and since then, no more than 80 cases have been reported. Despite distinct clinical characteristics, it is frequently misdiagnosed as onychomycosis partly due to remaining an unknown entity. The authors present the case of a patient with a 30-year history of nail abnormalities with more recent growth to alarming dimensions.