Large congenital melanocytic nevus rarely presents itself without hairs, with hardened skin and progressive depigmentation. We report a girl who presented with a large congenital melanocytic nevus in the left cheek. Over the years, the nevus became pruriginous, light brown, bumpy, and hard. Histology revealed nevus cells interspersed with dense fibrosclerotic collagen bundles. There are few reported cases of large congenital melanocytic nevus with this evolution, so-called desmoplastic hypopigmented hairless nevus.