Giant osteomas of the ethmoid and frontal sinuses ary very rare, with only a few dozen cases reported in the literature. Given their rarity, the clinical characteristics and treatment of this disease remain controversial. In this study, the clinical presentation and surgical methods used to treat three patients with giant osteomas of the ethmoid and frontal sinuses are described, combined with a review of the literature from 1975 to 2011. In total, 45 patients with giant osteomas arising from the ethmoid and frontal sinuses (including the present cases) have been reported in 41 articles. Headache and ocular signs are the most common symptoms. This disease often leads to intracranial or intraorbital complications. The main treatment for giant osteoma is surgery via an external approach. The outcome of surgery for giant osteoma is good, with rare recurrence, no malignant transformation and few persistent symptoms.