Primary carcinoma of the frontal sinus is quite rare, with an incidence of 0.3–1.0% of all paranasal sinus carcinomas. The early diagnosis is often difficult and the condition is often mistakenly considered to involve mucoceles, pyoceles or osteomyelitis. The present study reports the case of a 66-year-old male with squamous cell carcinoma originating in the frontal sinus. The presenting symptoms were a cutaneous nodule on the left side of the forehead and a gradually progressive headache. Magnetic resonance imaging (MRI) demonstrated erosion of the ethmoid sinus, frontal lobe and orbit. The radical resection under frontal craniotomy was performed followed by post-operative radiotherapy. Six months after the surgery, the MRI examinations did not find any recurrence, and the patient currently lives symptom-free. The present study illustrates that frontal sinus cancer should be diagnosed early with caution. Total surgical resection followed by radiotherapy and chemotherapy, used singly or in combination, may result in favorable outcomes. The current study discusses the diagnosis, treatment and prognosis of the present case and reviews the associated literature to emphasize the importance of an early identification of this rare disease.