Cholesterol granuloma (CG) is an expansile, cystic lesion most commonly observed in the temporal bone. CG is rarely encountered in the paranasal sinuses. Given its paucity in this region, the diagnostic and management aspects remain to be elucidated. Systematic review of the literature yielded 66 cases reported as individual data in 37 articles; an additional 69 cases were reported as aggregate data in 5 case series. The mean age of the 135 patients was 43.8 years, with a male:female ratio of 5.6:1. The most common presenting symptoms were orbital (66%), followed by headaches (19.3%). The most common location for CG was the frontal sinus (60%), followed by maxillary (34.1%) and ethmoid (3.7%) sinuses. Computed tomography (CT) imaging was used in 84 patients (63.3%), with bone erosion being noted in 80% of cases. Magnetic resonance imaging (MRI) was reported in only 9 cases (6.7%); high signal on T1- and T2-weighted images was evident in 77.8% and 66.7%, of cases respectively. Surgical data was available on 65 cases; drainage was achieved by open and endoscopic techniques in 52 (80%) and 13 (20%) cases, respectively. Primary symptom improvement and CG cavity patency was achieved in 92% of cases at a mean follow-up of 34.5 months. Paranasal sinus CG is most commonly observed in middle-aged males, arises in the frontal sinus, and typically presents with orbital symptoms. Both open and endoscopic techniques can be used with high success rate. The present study represents the first attempt in the literature to construct a clinical profile of this rare entity.