The concept of twin spotting (didymosis) explains the
combination of skin lesions in patients with Phacomatosis
We present the case of a 20-year-old patient with a
verrucous congenital plaque located on the right retroauricular
region diagnosed as sebaceous nevus associated
with Spillus nevus located contralaterally in the left
Phacomatosis pigmentokeratotica represents the association
of a organoid nevus with Spillus nevus differentiation.
In addition, muscular hemiatrophy, neurological
and ocular defects, deafness can be added to this association.