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Pachyonychia congenital is an infrequent genodermatosis, characterized by nail dystrophy, hyperkeratosis of the palms and soles, follicular keratosis and leukoplakia. It was seen in two male patients aged 12 and 35 years respectively. The younger patient had nail changes, palmo-planter keratoderma, eye changes, hypotrichosis and mental retardation, while the elder one had minimal nail changes, keratoderma and leukoplakia.
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