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Woolly hair is a congenital abnormality of scalp hair manifesting as short, kinked hair, which may also involve the hair over the other parts of the body. Keratosis pilaris has been a well known association of woolly hair, and can also be a part of the Naxos or Carvajal syndromes. We herein present a case of woolly hair with associated keratosis pilaris, canaliform dystrophy of nails, increased interdental spaces and recurrent bullous impetigo. Although keratosis pilaris and teeth abnormalities have been reported as isolated associations with woolly hair, such a combination of findings as seen in our patient has not been reported before.