Menkes' Kinky Hair Syndrome (MKHS) comprises an array of clinical manifestations including hair shaft abnormalities, epidermal hypopigmentation, and progressive cerebral degeneration that are transmitted as an X-linked recessive disorder affecting copper transport pathways in primarily young males. The oral manifestations of MKHS are scantly reported to include the presence of gingival enlargement and delayed eruption of primary teeth. The purpose of this report is to present a case of MKHS describing the intraoral clinical findings.