Timely genetic testing leading to early diagnosis of A‐T is crucial due to its plethora of implications on clinical management, particularly in those who develop malignancies. Thus, clinicians have to be astute in identifying diagnostic clues of A‐T.

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A 26-year-old Caucasian sailor, with no past medical history aside from headache for the last 1 week, was found dead in his cabin. The body was stored in a refrigerator on board and disembarked for autopsy 3 days later. Autopsy showed a large, nodular, necrotic and hemorrhagic retroperitoneal mass, and smaller hemorrhagic nodules in the brain, lungs, liver, and left kidney, with the brain being markedly edematous. Both testes were descended and normal. Histologically, the retroperitoneal mass showed a malignant mixed germ cell tumor comprising choriocarcinoma, embryonal carcinoma, and teratoma components. Retroperitoneal extragonadal germ cell tumors are uncommon, and this case of a young male who presented with headache and sudden death due to metastases is extremely rare.

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