There is only a small break in the front of the building from the roof to the ground. The narrator reveals that he is to stay in this house because his friend, Roderick, sent him a letter earnestly requesting his company. Roderick told the narrator in this letter that he was feeling bodily and emotionally ill, so the narrator rushed to his house. The narrator also mentions that the Usher family, while an ancient clan, never flourished. Only one member of the Usher family survived from generation to generation, so they were all in a direct line of descent without any siblings.
The inside of the house is just as spooky as the outside. The narrator makes his way through the long passages and to the room where Roderick is waiting. The narrator notes that his friend is paler and less energetic than he once was. Roderick tells the narrator that he suffers from nerves and fear. His senses are heightened. The narrator also notes that Roderick seems afraid of his own house. Further, Roderick's sister, Madeline Usher has taken ill with a mysterious illness that the doctors cannot even identify. The narrator proceeds to spend several days trying to cheer Roderick. He listens to Roderick play the guitar (and makes up words for his songs), he reads to Roderick, he sits with him for hours. Still, he cannot lift his sadness. Soon Roderick posits his theory that the house is unhealthy, just as the narrator had supposed at the beginning of the story.
Soon, Madeline dies, and Roderick decides to bury her temporarily in the tombs below the house. He wants to do this because he is afraid that the doctors might dig up her body for scientific examination (since her disease was so strange to them). The narrator helps Roderick put the body in the tomb. He notes that she has rosy cheeks, as some do after death. Roderick then confides to the narrator that he and Madeline were twins. Over the next few days, Roderick becomes even more uneasy.