Koënen tumor represents one of the main signs of the Tuberous Sclerosis, they are present in 50% of the patients with this illness. They are common ingrowings around the nail tissue which causes dysfunction in the nail. They are considered an hereditary variant of the fibrokeratoma which originates in the proximal nail fold or in the surrounding connective tissue. In the surgical approach, the tumor needs to be dissected up to its base, this requires the reflection of the proximal nail fold. The surgical procedure of a case is described and illustrated.
Koënen Tumor; hereditary fibroqueratoma; tuberous sclerosis