CS is characterized by a large range of systemic abnormalities [1,3]: patients usually have macrocephaly, trichilemmomas, and papillomatous papules/hamartomas [1]. CS is associated with a large range of PTEN hamartoma tumor syndrome (PHTS), which is a group of disorders characterized by the disorganized growth of native cells in native tissues [1]. The gene discussed is PTEN; the disease is Cowden syndrome 1.