BACKGROUND: Rhabdomyoma, the most common cardiac neoplasm in neonates. It commonly presents as mechanical obstruction of the outflow tract causing heart failure and arrhythmias. The neoplasm usually undergoes spontaneous resolution. Hence treatment should primarily be symptomatic, while surgical intervention is required in life–threatening situations. Half of these babies develop tuberous sclerosis. There are only few case series available describing its clinical profile especially from our country. Hence, we intended to analyze the same in our institute. AIM: The aim of our study was to analyze the cases of rhabdomyoma with neonatal presentation in our hospital. METHODS: A retrospective analysis of babies diagnosed as rhabdomyoma was done during the last 15 years (2003 – 2017), based on cardiovascular, radiologic and echocardiographic findings. RESULTS: Six neonates were diagnosed to have rhabdomyoma in the neonatal period during the last 15 years. Babies who were symptomatic were five (83%) [Two (33%) presented with cardiogenic shock & three (50%) presented with Supraventricular Tachycardia]. One baby was diagnosed based on fetal cardiac mass in antenatal ultrasound. The most common location was the left ventricle and in three babies the tumors were located in multiple chambers. Two (33%) babies had associated tuberous sclerosis. Five babies were managed medically and one required surgical intervention. Four (66%) babies were followed up and were found to be asymptomatic and thriving well. CONCLUSION: Rhabdomyoma commonest cardiac tumour in neonates is a self limiting condition. Though symptomatic in the newborn period management of low output cardiac failure and arrhythmias leads to better outcome.