Hematohidrosis is a mysterious and rare disorder characterized by one or more attacks of spontaneous, bloody sweating from intact surfaces of skin and/or mucous membranes. In the current literature, we faced a case of a 16-year old female who presented with recurrent attacks of right-sided bloody otorrhea upon exposure to extreme stress and anxiety. The patient had no history of bleeding disorders, trauma, or drug intake. Ear examination was completely normal with intact skin and tympanic membrane. All bloody investigations of the patient were within the normal range. The outcome was favorable in this case with medical treatment (beta-blocker and anxiolytics) and psychological support. In this article, we highlight with a detailed description of clinical characteristics and treatment options of this disease, followed by a review of the possible pathophysiological mechanisms. Our case together with previous …