The narrator received a letter from Roderick Usher, a childhood friend, requesting his company at his home due to him feeling ill. The narrator notices the house looks nearly as ill as Roderick himself.
Roderick's sister, Madeline, has also fallen ill and soon dies. Roderick decides to bury her below the house because he fears that the doctors might dig up her body to be examined. The narrator notices Madeline's cheeks are still rosy.
Over the next few days, Roderick becomes more uneasy. One night, the narrator cannot sleep. Roderick knocks on his door, hysterical. The narrator decides to read to Roderick in order to pass the night away.
The men hear noises that correspond to the story. They ignore the sounds. They become more distinct and they can no longer ignore them. Roderick reveals that he has been hearing these sounds for days and believes that they buried Madeline alive and that she is trying to escape.
Roderick yells that she is standing behind the door. The wind blows open the door and Madeline stands in white, bloodied, robes. She attacks Roderick and dies of weakness and Roderick dies of fear. The narrator flees the house.
After the last two members of the Usher family died, the house began to crumble and sink into the deep, dark waters of the marsh.