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Abstract: Congenital hypertrophy of the nail folds of the hallux describes an abnormality of the periungual soft tissues of the big toe characterized by hypertrophy of the nail fold which partially covers the nail plate and is frequently associated with inflammation and pain due to an ingrowing nail. We describe the clinical picture and follow-up of seven patients with this abnormality. In three patients the affected toe showed an asymptomatic, dome-shaped, hypertrophic lip that partially covered the nail plate. Four patients had acute inflammatory changes due to toenail ingrowth, with considerable swelling and reddening of the hypertrophic lip that was painful on pressure. Topical treatment with steroids was useful to reduce inflammation and produced persistent remission in two patients. Follow-up showed a spontaneous disappearance of the hypertrophic nail fold in one of the seven patients. In two patients the hypertrophic lip partially regressed, but remained clearly visible, while in two patients it remained unchanged. In two patients surgical correction of the soft tissue abnormality was necessary due to painful nail ingrowth unresponsive to topical treatment.