Two patients presented to the otolaryngology department of our university with a history of left nasal obstruction. The first case, a 50-year-old man, presented with a 3-month progressively worsening history of left maxillary swelling with mild facial pain and left nasal obstruction. A previous diagnosis of AIDS was reported; tuberous sclerosis was excluded by clinical workup. No alcohol consumption or smoking was reported. Nasal endoscopy revealed the presence of a reddish mass in the middle meatus, arising from the left inferior turbinate (Figure 1A). Narrow band imaging evaluation showed a relatively regular arrangement of intraepithelial capillary; the microvascular network and submucosal veins are nearly invisible (Figure 1B). The patient underwent a maxillofacial computed tomography (CT) scan with contrast that confirmed the presence of a soft density tissue of 13 mm in diameter, occupying the posterior portion of the middle turbinate, without evidence of bony erosion in the medial wall ( Figure 1C). The patient underwent an endoscopic resection of the mass. No signs of recurrence of the disease were observed 1 year after surgery.The second case, a 63-year-old male, presented with left-side nasal obstruction and rhinorrhea. Anterior rhinoscopy revealed in the left nasal fossa a smooth spherical nasal mass arising from the head of the left inferior turbinate, without bleeding on touch. Contrast-enhanced CT scan showed a soft density tissue, arising from the head of the left inferior turbinate, without evidence of bony erosion (Figure 2). These clinical features were suggestive of a benign tumor; thus, a complete excision with nasal endoscopy was performed. No signs of recurrence of the disease were observed 5 months after surgery.Both excised tumors consisted of vascular channels surrounded by thick muscular coats associated with intersecting fascicles of spindle cells. Cavernous-like vessels were also recognized in case 1 and aggregates of lymphocytes in case 2. Of note, mature adipocytes were identified in both tumors. While in case 1 they formed large aggregates, in case 2 they were distributed either singularly or in very small clusters. In both tumors, mitosis, pleomorphism, and necrosis were absent, and immunohistochemical analysis performed as described previously 1 demonstrated positivity of the spindle cells for smooth muscle actin and musclespecific actin and negativity for S100, HMB45, MelanA, estrogen receptor (ER) and progesterone receptor (PR); the proliferative activity (Ki67) was less than 3%, and Epstein-Barr Encoding Region (EBER) in situ hybridization was negative (Figure 3). Based on these findings, the pathologic diagnosis of sinonasal-ALM with adipocytic differentiation 2 was made.The occurrence of mature adipocytes in an otherwise typical sinonasal-ALM is not uncommon. Indeed, the term angiomyolipoma has been inappropriately used to describe typical sinonasal-ALM. However, angiomyolipoma is a completely different entity, since lesional cells co-express melanocytic (mainly HMB45) and my...