A 19-year-old man presented with a long-standing history of nasal obstruction, which gradually became worse over the past 2 years. Nasal endoscopy revealed a sizeable rounded mass covered by a normal-looking mucosa. Imaging studies showed a mass arising from the left middle turbinate that extended throughout the expanse of the anterior skull base. The tumour was resected via an endoscopic endonasal approach. Histopathological examination revealed a psammomatoid juvenile ossifying fibroma. The patient remains free of recurrence after almost 3 years of follow-up. Only four cases of ossifying fibroma with middle turbinate localisation have been reported in the literature so far, with our case representing the fifth and most extensive case. Clinical, radiological and histological findings should all be considered for establishing the correct diagnosis. An endoscopic approach represents an excellent therapeutic option. Long-term clinical and radiological surveillance is required due to the risk of recurrence.