We describe a patient who had bilateral, yellow papules of the upper eyelids. This proved to be the clinical manifestation of an atypical lymphoid hyperplasia of the orbits. We describe this clinical presentation as a new sign of this condition. This finding serves to broaden the differential diagnosis of yellow papules on the eyelids. Atypical lymphoid hyperplasia of the orbits poses a challenging problem. Their benign or malignant nature cannot usually be determined by clinical and radiologic criteria. Most of these infiltrates result in extraocular lymphoma. We describe a patient with bilateral, yellow papules of the upper eyelids that proved to be a manifestation of an atypical lymphoid hyperplasia of the orbits.