Ataxia telangiectasia (AT) syndrome is a rare, multisystemic autosomal recessive disorder. Ataxia is the first noticeable sign, while telangiectasias usually become evident after the age of 6 and are present in almost all cases. Other skin manifestations reported in these patients are premature aging with graying of the hair. The diagnosis of AT can be challenging and can be made by combination of neurological signs with other clinical characteristics, such as telangiectasias. The typical dermatoscopic morphology of telangiectasias, consisting of wave-shaped dilated vessels, can help in the diagnosis of this complex disease.