Ethmoid mucocele, a condition that usually affects adults, can be found in both children and infants with cystic fibrosis. We have cared for two patients with cystic fibrosis and ethmoid mucoceles. Because we could find no references to the prognosis or any long-term follow-up data for this disorder, we contacted the physicians and families of six of the seven patients with this condition previously reported in the English literature to determine how they were managed surgically and the incidence of recurrence. Regardless of the mode of therapy, none of the mucoceles recurred.