To evaluate the demographic, clinical, and histopathologic characteristics of periocular inverted follicular keratosis (IFK), a very rare lesion with poorly defined characteristics.Retrospective case series.We evaluated 11 patients with clinically diagnosed IFK confirmed by histologic analysis. Data were collected on the patients' demographics, clinical presentation and course of the disease, signs and symptoms, location of the lesion, and outcomes of treatment.The patients' mean age was 71 years (range, 32-91 years). Seven (64%) of the patients were female. Eight of the patients (72.7%) had no symptoms, two (18.2%) reported itching, and one (9.1%) had edema and bleeding of the lesion. The lesion affected the upper eyelid in 4 of the patients (36%), the lower lid in 3 of the patients (27%), and the inner canthus in 4 of the patients (36%).IFK has no specific clinical characteristic and thus requires histologic confirmation for its diagnosis and appropriate management.