Cardiac rhabdomyoma (CR) is a rare tumor commonly associated with tuberous sclerosis. They are often detected prenatally or in early infancy. The case of a Nigerian human immunodeficiency virus (HIV)-exposed neonate with CR who presented with supraventricular tachycardia and cardiovascular collapse is presented. The infant was born to a mother on highly active antiretroviral therapy (HAART). The possible role of HIV and HAART in CR etiology and the difficulty in the management of this case are highlighted.
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