Background and objectiveHair-thread tourniquet syndrome (HTTS) is a rare and potentially dangerous condition that occurs when a hair strand or fabric thread is wrapped around the penis, clitoris, toes, fingers, or other appendages, leading to focal edema, ischemia, and necrosis. This study aimed to examine the cases of six female patients with isolated clitoral hood HTTS.MethodsThis was a retrospective study involving six female patients (age range: two to six years) with isolated clitoral hood tourniquet who presented to the outpatient department (OPD) and emergency room (ER) of the pediatric and adolescent gynecology service at King Abdulaziz University Hospital, Jeddah, Saudi Arabia between January 2010 and December 2021. Data related to patients’ clinical presentation, symptom duration, and management were recorded.ResultsThe most common symptom of isolated clitoral hood HTTS in all six cases was pain, followed by redness, itching, discomfort while sitting, and dysuria. Local signs included edema in three cases, tight hair tourniquet around a portion of the clitoral hood in four cases, and loose hair tourniquet around a portion of the clitoral hood in two cases, one of which was during the first episode in a patient who had recurrence (Case 1). Sedation and local anesthetic were used in five cases involving the local removal of four hairs and a strangulated skin. This was followed by local care and antibiotic ointment application. The edges were closed by interrupted stitches in two of the cases as the resulting wounds were broad. Limited clitoral unhooding was performed under general anesthesia in one patient, who had repeated bouts of autoamputation of parts of the hood tissue with resulting disfiguration of the remaining redundant hood, to avoid the additional risk of organ loss. Only two patients experienced recurring episodes.ConclusionsA high index of suspicion should be maintained when encountering these patients, which can facilitate a prompt resolution to save the affected tissue. HTTS should be suspected in all females presenting with a prominent clitoral hood, genital pain, structural abnormality, swelling, or discomfort. This is the first study to report isolated clitoral hood HTTS; it describes the presentation, manifestation, and management of patients with HTTS. It also addresses preventive strategies to alert mothers and treating physicians of the pediatric and adolescent-age group females to the defect and its underlying causes, particularly when patients have genital structural abnormalities such as excessive clitoral hood labial hypertrophy or ambiguous genitalia.