Aim: To determine the characteristics of isolated ventricular septal defect (VSD) in association with infective endocarditis (IE) among children with isolated VSD and its outcome. Method: This was cross-sectional study. Data was collected prospectively from infective endocarditis patients with isolated ventricular septal defect (VSD), admitted in pediatric cardiology ward of The Children’s hospital and Institute of Child Health, Lahore. Study period was from September 2018 till March 2021. The data was analyzed by using SPSS-26. Result: Sixty patients of ventricular septal defect (VSD) with infective endocarditis (IE), fulfilling the inclusion criteria, were included in the current study. Mostly about 60% were male. Transthoracic Echocardiography was done for all the patients. Single vegetation was found among 68.3%, rest were having multiple vegetations. seen. Approximately 57% were having small size vegetations while remaining 43% were with moderated – large vegetations. Complications were reported in only 18% of the study population. Regarding outcome majority of the patients (90%) were successfully treated with medical management alone, while only 8.3% required surgical intervention as well. Only 1 out of 60 patients included in the study was expired. Characteristic of VSD, size of vegetation, and number of vegetations have significant influence on development of complications (p = <0.05). Conclusion: Risk of complications among infective endocarditis with isolated VSD is comparatively greater with large vegetation and multiple number of vegetations as compared to single or small vegetation. Medical management is main stay of treatment while surgical intervention is usually required among infective endocarditis patients with DCSA-VSD & subaortic-VSD. Key words: Ventricular septal defect (VSD), DCSA (doubly committed sub-arterial) VSD, Infective endocarditis (IE).