Tuberous sclerosis is a neurocutaneous syndrome involves skin, brain, kidneys, heart and eyes primarly. Cardiac rhabdomyomas accompany to tuberous sclerosis frequently. Congenital adrenal hyperplasia is group of autosomal disorders of adrenal cortex characterized with enzyme deficiency at cortisol biosynthesis. There is only one case with tuberous sclerosis and congenital adrenal hyperplasia reported in literature. We report a case with intracardiac masses which were detected in the left ventricle with prenatal and postnatal echocardiography. Tuberous sclerosis with subependymal nodules and cortical tubers was detected at postnatal magnetic resonance imaging. Also congenital adrenal hyperplasia was determined at the infant which had areolar and scrotal hyperpigmentation.

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