A child with a stroke, drug-refractory epilepsy and congenital heart disease: can a hemispherectomy be safely performed between staged cardiac procedures? Abstract Background The safety of hemispherectomy between staged cardiac procedures is unknown and not previously reported. Method Retrospective review of a case with drug-resistant epilepsy due to stroke following bidirectional cavopulmonary connection (BDCPC). Results This report describes the first case of a successful pediatric peri-insular functional hemispherectomy in the setting of a BDCPC. A discussion of the complex preoperative planning from both a cardiac and neurological perspective is presented. Considerations regarding hemispherectomy and its effects on the cardiac physiology, and perioperative considerations are emphasized in clinical decision making. Conclusions A multidisciplinary approach was critical in this child which led to a successful outcome.