A solitary fibrous tumor (SFT) is a benign neoplasm, firstly described as a mesenchymal tumor of the pleura. Its incidence range in the head and neck region is about 5–27%, but only rarely does it affect paranasal sinuses. The differential diagnosis is challenging, owing to its erosive growth pattern and immuno-histochemical features. SFTs have an aggressive behavior and an important recurrence potential. Therefore, a radical surgical excision is the gold standard therapeutic procedure. A rare SFT originating from the right maxillary sinus is reported here. The 37-year-old patient presented to the outpatient clinic with a painful expansive lesion in the whole right maxillary region. The overlying skin was inflamed and the patient had no epistaxis episodes. The 1.5 dentary element tested negative for vitality; however, a puncture of the lesion led to a hematic spill and no purulent discharge. An endoscopic-guided biopsy was suggestive either of SFT or hemangioperictoma, excluding a malignant neoplasm. A multi-equipe surgical team was activated. The lesion was embolized in order to achieve a good hemostatic control and, after 48 h, the neoplasm was radically excised with a combined open and endoscopic approach. The patient was disease-free at 12-month radiological and clinical follow-up. Given the rarity of this lesion and the delicacy required in addressing head and neck neoplasms, we believe that the present case report might be of help in further understanding how to approach cranio-facial SFTs.