Background Respiratory epithelial adenomatoid hamartoma (REAH) is a benign lesion of the sinonasal tract that may mimic more concerning pathology. Clinical factors associated with REAH have not been well characterized. Objective To report our findings on patients with this pathologic diagnosis. Methods A retrospective chart review of patients with REAH between September 2006 and November 2019 was conducted. Data collected included clinical allergic rhinitis and asthma history, additional sinonasal diagnoses, prior sinus surgery, and the location of the REAH within the sinonasal cavity. Results Twenty-six patients were identified (53.8% male, mean age 62 years [range, 29–93]). Bilateral REAH occurred in 50%. REAH was located at the superior nasal septum in 84.6% cases, with the remainder identified in sinus contents submitted for pathology, making definitive site uncertain. Concurrent sinonasal inflammatory disorders were identified in 18 patients (69.2%), including chronic rhinosinusitis with nasal polyps—not otherwise specified (6), chronic rhinosinusitis without nasal polyps (4), aspirin-exacerbated respiratory disease (2), allergic fungal rhinosinusitis (1), central compartment atopic disease (5), and IgG4-related sclerosing disease (1). Eight patients had isolated REAH. Adequate allergy records were available for 19 patients, of which 18 of 19 (94.7%) had clinical allergic rhinitis. Conclusions REAH is a benign sinonasal lesion commonly located within the central compartment of the nasal cavity, a site of significant allergen exposure. Affected patients have a high incidence of allergy along with chronic inflammatory conditions. The coexistence of REAH within inflammatory nasal mucosa in a consistent anatomic location, suggests REAH may have a similar etiology to central compartment atopic disease, with resultant respiratory glandular ingrowth within long-standing reactive changes of mucosa derived from ethmoid embryologic origin.