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Uhl's disease is a rare disorder secondary to the uncontrolled destruction of right ventricular myocytes during the perinatal period. We present here the case of a 1-month-old child who died suddenly of Uhl's disease, which was only diagnosed at autopsy and histological examination. From an anamnestic point of view, the child's sister had also died at about 1 month of age from the same pathology.

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