Background/Aim: Sinonasal rhabdomyosarcoma (RMS) is a rare soft tissue malignancy. Due to the limited cases, the clinicopathological features and prognostic factors are still not well understood. Patients and Methods: This retrospective review included eight patients with sinonasal RMS at our institution between 2004 and 2020. Patient demographics, tumor features, Intergroup Rhabdomyosarcoma Study Group (IRSG) stage and clinical group, treatment strategy, and survival rates were evaluated. Kaplan-Meier analysis and log-rank tests were performed to analyze the possible prognostic factors. Results: We observed a predominance of male sex and alveolar-type tumor in sinonasal RMS. Nasal obstructions and neck masses were the most common symptoms. Patients with pretreatment lactate dehydrogenase (LDH) levels >400 U/l and negative immunohistochemical staining for desmin or MyoD1 had lower survival rates. Conclusion: In patients with sinonasal RMS, pretreatment LDH levels >400 U/l and negative immunohistochemical staining for desmin or MyoD1 may suggest a poor prognosis. These factors can not only contribute to the prediction of prognosis in patients with sinonasal RMS but also facilitate the design of more appropriate treatment strategies.Rhabdomyosarcoma (RMS) is a malignant tumor with a propensity for skeletal muscle differentiation. It is the most common soft tissue sarcoma in children, accounting for approximately 50% of all pediatric soft tissue sarcomas (1-3). However, it is rare in adults, accounting for <1% of all adult malignancies. RMS can be classified into four histological subtypes: embryonal (ERMS), alveolar (ARMS), pleomorphic (PRMS), and spindle cell/sclerotic. ERMS is the most common type, followed by ARMS (4, 5). ARMS is more aggressive than the other subtypes and is often associated with a worse prognosis. ARMS can be further classified according to the presence or absence of the paired box (Pax) proteins 3 and 7/forkhead box O1 (PAX3/7-FOXO1) fusion gene. The outcome in patients with fusionnegative ARMS is indistinguishable from that in ERMS and differs significantly from that in ARMS with fusion genes (6).Approximately 35% of RMS cases arise in the head and neck region, including the orbital, parameningeal, and nonparameningeal areas (3). Tumors in the parameningeal areas have the worst outcome among those in the head and neck region, especially the paranasal sinuses, owing to the structural complexity and tendency for central nervous system (CNS) and skull base invasion (3,7,8). As the tumor location affects the disease prognosis, it appears alongside the usual TNM in the Intergroup Rhabdomyosarcoma Staging Group (IRSG) staging of RMS (9). In addition, the IRSG clinical group for RMS is based on the extent of residual disease after resection and evidence of regional lymph node (LN) or distal metastasis, which is one of the most important prognostic factors in RMS. The treatment strategy for RMS, which includes surgery, chemotherapy, and radiotherapy, is risk-based. The main treatment strategy for si...