An unnamed narrator approaches the house of Usher on a "dull, dark, and soundless day." This house—the estate of his boyhood friend, Roderick Usher—is gloomy and mysterious. The narrator observes that the house seems to have absorbed an evil and diseased atmosphere from the decaying trees and murky ponds around it. He notes that although the house is decaying in places—individual stones are disintegrating, for example—the structure itself is fairly solid. There is only a small crack from the roof to the ground in the front of the building. He has come to the house because his friend Roderick sent him a letter earnestly requesting his company. Roderick wrote that he was feeling physically and emotionally ill, so the narrator is rushing to his assistance. The narrator mentions that the Usher family, though an ancient clan, has never flourished. Only one member of the Usher family has survived from generation to generation, thereby forming a direct line of descent without any outside branches. The Usher family has become so identified with its estate that the peasantry confuses the inhabitants with their home.

The narrator decides to read to Roderick in order to pass the night away. He reads "Mad Trist" by Sir Launcelot Canning, a medieval romance. As he reads, he hears noises that correspond to the descriptions in the story. At first, he ignores these sounds as the vagaries of his imagination. Soon, however, they become more distinct and he can no longer ignore them. He also notices that Roderick has slumped over in his chair and is muttering to himself. The narrator approaches Roderick and listens to what he is saying. Roderick reveals that he has been hearing these sounds for days, and believes that they have buried Madeline alive and that she is trying to escape.