<p>Perinatal arterial ischemic stroke (PAIS) is one of the frequent causes of mortality and morbidity, but its etiology remains unclear. <em>COL4A1 </em>and <em>COL4A2</em> mutations are monogenetic causes of weakness of the basement vascular membranes resulting in cerebral small-vessel disease, cerebral hemorrhage, and porencephaly. We hypothesized that variations in the&nbsp;<em>COL4A1</em>&nbsp;and&nbsp;<em>COL4A2</em>&nbsp;genes cause PAIS and performed mutation screening of these genes in 17 PAIS patients by whole-exome sequencing. Clinical, demographic, and laboratory data of the 17 PAIS patients were obtained by evaluating hospital files retrospectively. Patients included in the study were invited to the clinic for&nbsp;<em>COL4A1</em>&nbsp;and&nbsp;<em>COL4A2</em>&nbsp;mutation analysis. Results: The patient group consisted of 13 females (76.5%) and four males (23.5%) with a mean age of 107.4 ± 11.5 months. Maternal/fetal and prothrombotic risk factors identified in 52.9% and 94.1% of the patients, respectively. Whole-exome sequencing analysis did not reveal&nbsp;<em>COL4A1</em>&nbsp;and&nbsp;<em>COL4A2</em>&nbsp;pathological mutations in any of the patients. &nbsp;Although we did not find an association between PAIS and&nbsp;<em>COL4A1</em>&nbsp;and&nbsp;<em>COL4A2</em>&nbsp;variations, we believe that new studies with larger patient populations may reveal such a relationship.</p>