First introduced by Michaëlsson in 1981, Rombo syndrome is an extremely rare disorder with less than ten confirmed cases worldwide (Ashinoff et al. 1993; Michaëlsson et al. 1981; van Steensel et al. 2001). The prevalence of Rombo syndrome is estimated to be less than one in a million (Michaëlsson et al. 1981). It is characterized by hypotrichosis, trichoepitheliomas, along with vermiculate atrophoderma, and peripheral vasodilation with cyanosis (Ashinoff et al. 1993; Michaëlsson et al. 1981; van Steensel et al. 2001). Skin changes appear at ages 7–10 (Michaëlsson et al. 1981; van Steensel et al. 2001). Acral erythema, cyanosis, and redness in lips and hands were documented, along with follicular atrophy on the exposed face areas (Michaëlsson et al. 1981). Milia and telangiectatic vessels develop later in adulthood (Ashinoff et al. 1993; Michaëlsson et al. 1981; van Steensel et al. 2001). The skin atrophy deteriorates, leading to vermicular atrophoderma. Abnormal hair loss in eyelashes and eyebrows has also been observed in adulthood. Trichoepitheliomas were also observed in a case (Michaëlsson et al. 1981).