A case of neonatal hypertrophic cardiomyopathy (HCM) without obstruction is reported. Von Recklinghausen neurofibromatosis in the mother and several relatives and the occurrence of café-au-lait spots in the child at 6 weeks of life led to the association of HCM with this phacomatosis. The spontaneous regression of the myocardial hypertrophy after 6 weeks makes this first report of neonatal HCM in the course of Von Recklinghausen neurofibromatosis peculiar. Several reports in the literature mention the possibility of such a spontaneous regression of HCM or diffuse cardiac tumors, especially in the course of phacomatosis.

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