Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 7 5 2023 02 12 216 220 Alireza Golbabaei Department of Perinatology and Fetal Cardiology, Shariati hospital,Tehran University of Medical Sciences, Iran. Mahsa Naemi naimi_mahsa@yahoo.com maasoumeh saleh Department of Obstetrics and Gynecology, Shariati Hospital, Tehran University of Medical Sciences, Iran. 2021 04 30 2021 06 16 Double inlet left ventricle (DILV) is a rare congenital cardiac malformation that is defined as an anomaly with univentricular atrioventricular (AV) connection, and single ventricular morphology. This variation can be associated with the inlet and outlet cardiac abnormalities. In the current report, we present a case of Holms heart, as a rare variant of double-inlet left ventricle. https://crcp.tums.ac.ir/index.php/crcp/article/view/525