A persistent LSVC with an absent right superior vena cava (RSVC) is unusual, encountered in about 10% of patients with persistent LSVC. We present 14 patients with a prenatal diagnosis of single LSVC and situs solitus, including prenatal findings and information on medium-term follow-up. We identified patients with situs solitus and a persistent LSVC born between March 2004 and March 2020, which had been diagnosed prenatally between December 2003 and November 2019. From this cohort, we identified those with absent RSVC. In the population of women undergoing fetal echocardiography, the prevalence of persistent LSVC in situs solitus was 0.43% (84/19,712). For the 84 identified patients and for the entire population respectively, 14/84 (17%) and 14/19,712 (0.07%) had a single LSVC (absent RSVC). Of 14 patients with a single LSVC, 8 (57%) were male. For the 84 identified patients and for the entire population respectively, 70/84 (83%) and 70/19,712 (0.36%) had bilateral SVCs. For a single LSVC (absent RSVC) and situs solitus, the majority had no associated cardiac, extracardiac, or syndromic abnormalities.