Introduction: Juvenile xanthogranulomatosis is a rare disease that commonly occurs in early childhood. Adults can be rarely affected. The cutaneous manifestation occurs in most cases, as a solitary, indurated red-yellow nodule. It usually occurs at the head and neck level, followed by the trunk, lower and upper extremities. Although infrequent, extracutaneous manifestations can present with main ophthalmological involvement. Objective : To describe a rare case of juvenile xanthogranulomatosis in the external auditory canal. Clinical Case report : This rare case is reported due to its unusual location. This is a 42 years old male patient, who came to consultation due to the appearance of a lesion in the right external auditory canal with progressive increase in size associated with intermittent serohematic otorrhea and hearing loss. The diagnosis was made by clinical, histopathological and immunohistochemical findings. Total excision of the lesion was performed. Later, he had adequate evolution and improvement of symptoms. Conclusion : Adult xanthogranulomatosis is a rare disease, with clinical findings that may suggest multiple differential diagnoses, thus histopathological and immunohistochemical support are important to guide the diagnosis.