A 4-year-old girl presented with nevoid hypermelanosis since birth, exhibiting hyperpigmented macules that gradually spread in a whorled pattern following Blaschko's lines from her C3 to C6 dermatome. She also showed alterations in hair color, texture, and form beginning at age 1 in well-delimited occipital regions, with one patch of tightly curled hair differing from surrounding scalp hair. This case report describes the association between wooly hair nevus and nevoid hypermelanosis in a young female patient.