Spontaneous cervical arterial dissection is an important cause of juvenile stroke. However, etiopathology and genetic background remain poorly understood. We report on a 45-year-old-male patient with homozygous alpha-1-antitrypsin (alpha-1-AT) deficiency in whom internal carotid artery dissection occurred in the absence of any other known risk factors. The relevance of alpha-1-AT deficiency for spontaneous cervical arterial dissections is discussed.