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Woolly Hair with Complete Atrioventricular Dissociation: A Rare Association

Woolly hair is a rare congenital abnormality of structure of the scalp hair characterized by tightly coiled hair involving part or the entire scalp. There are mainly two types of woolly hair; autosomal dominant/hereditary woolly hair and autosomal recessive/familial woolly hair. We hereby report two cases of autosomal recessive/familial woolly hair from a single family associated with complete atrioventricular dissociation in one sibling, which is a very rare association and only a single case has been reported in literature with this association. This case report signifies the importance of cardiac evaluation in woolly hair and stresses the fact that hair examination is not just cosmetic but can be lifesaving.