Basal Cell Carcinoma Arising from an Epidermal Naevus

Epidermal naevus is a congenital cutaneous hamartoma with a benign course.  We highlight  a  rare  case  of  epidermal  naevus  with  concurrent  basal  cell  carcinoma. A 79-year-old male had a skin biopsy at our centre for an enlarging skin nodule within a linear papular lesion measuring 2 x 4 cm at the left side of his neck, which was  later  diagnosed  as  basal  cell  carcinoma  arising  from  an  epidermal  naevus.  PIK3CA  mutation  is  attributed  to  basal  cell  carcinoma  which  suggests  the  basal cell  carcinoma  component  is  independent  of  the  epidermal  naevus  component.  Clinicians and pathologists must be aware of possible malignant changes that might arise in an epidermal naevus.