AIM: Myopericytoma (MPC) is a rare tumour characterized by a perivascular proliferation of pericytic cells with myoid differentiation and a typical spindle shape. Except for the rare malignant cases, MPC mostly shows a benign course. Symptoms are often non-specific, and the diagnosis could be accidental. Simple biopsies are often non-diagnostic and do not provide any information about the benign or malignant course of the disease. General agreement for its management is lacking. CASE PRESENTATION: An old patient was referred to our tertiary cancer centre for left nasal obstruction for the previous three months. No worker risk factors were reported. The nasal endoscopy with enhanced endoscopic systems equipped with digital post-processing image enhancement technology (I-SCAN) and Narrow Band Imaging (NBI) revealed a non-bleeding reddish mass located at the anterior third of the left nasal fossa floor, about 1 cm in size and posteriorly a second more minor similar lesion at the level of the nasal septum. The patient underwent a radical endoscopic excision with diagnostic and curative intent. RESULTS: No significant intra-peri- and postoperative complications were recorded. The final histopathological exam revealed a double synchronous sinonasal myopericitoma removed with safe margins. The patient is still alive with no evidence of disease after three years from surgery. CONCLUSIONS: MPC is a challenging disease that must be considered in the differential diagnosis of all the vascular lesions of the head and neck region. Surgery should be performed with radical margins to provide a definitive cure. The endoscopic approach may allow a radical removal with a low risk of surgical complications, allowing the possibility of removing representative material for an accurate histopathological diagnosis.