El narrador, Egaeus, se prepara para casarse con su prima, Berenice. Egaeus sufre extraños ataques de ensimismamiento durante los cuales parece aislarse por completo del mundo exterior. Berenice empieza a deteriorarse debido a una enfermedad desconocida, hasta que la única parte de su cuerpo que parece permanecer viva son sus bonitos dientes, con los cuales Egaeus empieza a obsesionarse. Berenice muere finalmente y él entra en uno de sus trances. Un criado lo interrumpe informándole de que la tumba de Berenice ha sido profanada. Egaeus se descubre cubierto de sangre, y a su lado, diversas herramientas de dentista y una cajita conteniendo 32 dientes ("thirty-two small, white and ivory-looking substances"). Por otra parte, todo hace pensar que Berenice fue enterrada viva.ogers.
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