<i>Background:</i> Congenital infections are associated with a wide spectrum of clinical symptoms, including lenticulostriate vasculopathy (LSV). <i>Objective:</i> To determine the relationship between LSV and congenital infections, as diagnosed by TORCH serology and viral culture for cytomegalovirus (CMV). <i>Methods:</i> All neonates with LSV admitted to our neonatal intensive-care unit from 2004 to 2008 were included in the study. Results of maternal and neonatal TORCH testing were evaluated. <i>Results:</i> During the study period, cranial ultrasound scans were performed in 2,088 neonates. LSV was detected in 80 (4%) neonates. Maternal and/or neonatal serological TORCH tests were performed in 73% (58/80) of cases. None of the mothers or infants (0 of 58) had positive IgM titres for <i>Toxoplasma</i>, rubella, CMV or herpes simplex virus. Additional urine culture for CMV was performed in 38 neonates. None of the infants (0 of 38) had a positive CMV urine culture test. <i>Conclusions:</i> Routinely applied efforts to diagnose congenital infections in cases presenting with LSV have a poor yield. Routine TORCH screening in neonates with LSV cases should only be regarded as mandatory once well-designed studies demonstrate a clear diagnostic benefit.