Paranasal sinus mucoceles are benign, expansive, cystic lesions which arise from retention of mucus secretions. They arise primarily in the setting of cystic degeneration of the sinus mucosa or secondarily in the setting of obstruction of the sinus ostium due to mucosal trauma or chronic inflammation. Septal mucoceles are rare. Only 10 cases to our knowledge have previously been reported. Patients are exclusively male, in their middle years, usually with a history of nasal trauma or nasal surgery. Nasal obstruction followed by headache present for 3 months to a year are the most common presenting symptoms. Here, we present a case of septal mucocele with dystrophic bone formation in a 57-year-old man arising 35 years after open septorhinoplasty treated successfully with total excision via an endoscopic, endoseptal approach. A review of the available literature was conducted to provide a consolidated update on diagnosis and management.