William reads a letter he has received from his long lost friend, Roderick Usher. Roderick asks William to visit him. William makes the journey to The House of Usher. As a gift, he brings Roderick an antique music box. The butler takes William into Roderick’s study, and for the first time since they were children, the two men meet. William shows Roderick the gift he brought, but the sound of the music box causes Roderick great distress. Roderick reveals that his sister Madeline has become extremely ill and that she is confined to her bed upstairs. William promises to do all he can to help. Later that night, William is visited by horrible nightmares featuring unspeakable visions of Roderick, Madeline, and the House of Usher. He awakes in a panic. The next day, William finds Roderick in his study. Still shaken from his dreams, William attempts to make conversation. William inquires about Madeline’s health, and Roderick quietly informs him that Madeline passed away in the night.
William, the doctor, and the butler, assist Roderick in burying Madeline. As the lid to the coffin is being shut, William realizes that the brother and sister are twins. Over the next few days, Roderick becomes more and more agitated. In a fit of anger, he throws the butler and the doctor out of the house. Only William and Roderick remain. That night, a storm begins. To pass the time in the study and ride out the storm, William recites a story they both knew as children. The story concerns a brave knight that breaks into a hermit’s cave seeking shelter from a storm, but instead finds a dragon guarding a stash of gold. William is repeatedly interrupted by the raging storm and also by constant pounding and scratching noises from outside. As William looks toward the door, Madeline appears, looking ghastly after clawing herself out of her own grave. She lunges toward Roderick, falling violently upon him. The house itself begins to crumble. William runs for his life as the house suddenly falls in on itself.