We report the first case of life-threatening extreme neonatal-acquired methemoglobinemia that occurred during inhaled nitric oxide (iNO) at the standard 20 ppm dose in a neonate with early onset sepsis and suprasystemic pulmonary hypertension. Life-threatening methemoglobinemia has been efficaciously treated with methylene blue and ascorbic acid, while stopping iNO and starting iloprost and sildenafil. The patient was subjected to various tests (including gene sequencing and hemoglobin electrophoresis) and did not have any known genetic cause or predisposition for methemoglobinemia. Neuroimaging and the 2-year clinical follow-up were completely normal.

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