We present 45 patients with angiomatoid polyps of the nasal and paranasal regions (APNPRs), which are underrecognized lesions which may cause considerable diagnostic difficulties. There were 32 men and 13 women in our series. The average age at diagnosis was 49 years in men and 54.3 years in women. Locations were known in 41 cases and included the nasal septum (14), maxillary sinus (12), ethmoid sinuses (5), lateral wall of the nasal cavity (5), sphenoid sinus (1), and nasal cavity, not otherwise specified (4). X-ray or computed tomography was performed in 19 cases and revealed bone erosions/deviations in four cases. Initial misdiagnoses submitted by referring pathologists were reported in 20/32 of the consultation cases. Our study confirms that APNPRs are benign lesions which often recur and sometimes multiple recurrences are seen. APNPRs sometimes cause severe changes of the skeletal bones especially in recurrent lesions. Awareness of the above described features and familiarity with the clinical presentation of APNPRs is the best way to avoid a misdiagnosis.