Mai de Meleda (keratoderma palmoplantaris transgrediens) is a rare autosomal recessive form of palmoplantar keratoderma characterized by hyperkeratosis of the palms and soles which appears soon after birth and progressively (progrediens) involves other areas (transgrediens) of the body skin. We report the case of a patient with mal de Meleda with three unusual facultative features, i.e. prominent knuckle pads, peculiar finger-nail anomalies and pseudo-ainhum on both fifth fingers. Four other members of the family are also affected by mal de Meleda.