Primary carcinomas of the sinonasal tract are uncommon malignancies in the head and neck. First reported in the in literature in 2014, SMARC-B1 deficient sinonasal carcinomas is an aggressive variant of sinonasal malignancies characterized by loss of expression of SMARCB1 (INI), which is a vital component of the SW1/SNF complex. Due to its peculiarity and the histopathologic properties shared with other sinonasal malignancies, along with limited treatment guidelines, SMARCB1 deficient tumors own significant therapeutic and diagnostic challenges. In this report we describe the first case of SMARCB1 deficient sinonasal carcinoma in Lebanon, it is a case of a 51-year-old male patient, presented with sensation of fullness in his nostrils and night snoring. Investigations done showed a large a mass which was excised and surgical pathology showed SMARCB1 deficient sinonasal carcinoma confirmed by immunohistochemistry. This report sheds the light on the diagnostic challenges and aggressive nature of SMARCB1 deficient sinonasal tumors. Given the lack of appropriate number of cases to establish guidelines for management on this topic we aim to provide a comprehensive review of the available literature in order to enhance understanding of this complex malignancy and provide insights for future directions.