Cholesterol granulomas are typically identified by their histological features, including cholesterol crystals, giant cells, fibrosis, and inflammation. They occur predominantly in the middle ear, petrous apex, and orbital region, with rare occurrences in the labyrinth. Diagnosis of these lesions is challenging due to their imaging similarities with endolymphatic sac tumors, particularly in preoperative differentiation. In the present case, a 60-year-old woman diagnosed with an endolymphatic sac tumor through preoperative magnetic resonance imaging underwent a transmastoid surgical procedure, and subsequent postoperative histopathological analysis confirmed a cholesterol granuloma. We report this rare case of granuloma confined within the labyrinth, highlighting the importance of radiological and histopathological diagnoses in determining the appropriate therapeutic approach.