Abstract
BACKGROUND: Myelolipomas are benign tumors composed of mature fat cells and hematopoietic cells. While they are most commonly found in the adrenal glands, extra-adrenal myelolipomas (EAMs) can occur. There are very few cases of EAMs occurring in the petroclival region reported. OBSERVATIONS: The authors present the case of a 77-year-old woman undergoing evaluation for submandibular swelling and discomfort, who was incidentally found to have a petroclival lesion on imaging. She also reported hearing and sensory deficits. The patient was initially suspected to have a benign lesion or cholesterol granuloma. She underwent endoscopic resection, and histopathological analysis ultimately confirmed the diagnosis of a primary intracranial EAM. LESSONS: This case underscores the importance of considering EAMs as part of the broad differential diagnosis for petroclival lesions and highlights the diagnostic challenges posed by rare skull base entities. Given their radiographic similarities to more common pathologies, effective management requires multidisciplinary collaboration across radiology, pathology, surgery, and primary care teams. https://thejns.org/doi/10.3171/CASE25377.