A 28-year-old Costa Rican woman presented with a 6-year history of an asymptomatic progressive localized livedo racemosa on her limbs. Histological examination revealed a lymphocytic vasculitis targeting the arterioles in the deep dermis. In addition, a distinct hyalinised fibrin ring was noted at the periphery of the vessel lumen. These findings were consistent with the recently described entity known as lymphocytic thrombophilic arteritis. An extensive array of investigations did not show any underlying systemic disease, and the patient has remained in good health without treatment.