Background: Investigation of the maternal cause of death and pregnancy-related death is one of the most important responsibilities of a forensic pathologist. From the public health point of view, it may help to prevent losses during and following pregnancy and save the lives of women, especially in developing countries. Methods: We report a case of maternal death with a history of neurofibromatosis type 1 who presented asymptomatic and normotensive with normal laboratory test results. Results: The first attack after delivery was associated with pulmonary edema, which led to death. Investigation during the medico-legal autopsy discovered a left suprarenal tumor with the diagnosis of pheochromocytoma, which was confirmed by further histopathology testing. Conclusion: We believe that although the association of neurofibromatosis type 1 and normotensive pheochromocytoma during pregnancy has been reported rarely, the possibility must be considered for evaluation before elective operations to adopt proper preoperative protocols.
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