Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a newly defined, rare malignant tumor of the nasal cavity. The clinical course and response to treatment remain uncertain. The purpose of this study is to report a new case of SNRCLA and review the literature to determine clinical characteristics, treatment options, and outcomes. A 26-year-old male presented with headache, epistaxis, and nasal obstruction. Physical examination revealed a tumor involving bilateral ethmoid sinuses and MRI revealed extension through the cribriform plate. Surgical excision with endonasal and a bifrontal craniotomy was performed followed by adjuvant radiotherapy (RT). After RT, the patient had persistent disease requiring salvage surgery. There are few previously reported cases of SNRCLA. A literature review yielded 14 previously reported cases with convincing diagnostic evidence of SNRCLA. Common presenting symptoms were epistaxis and nasal obstruction. Surgical excision was the primary treatment in fourteen cases, nine received RT, and none received chemotherapy. However, three cases had persistent or recurrent disease. Surgical excision is the mainstay of treatment for SNRCLA and adjuvant RT has been used in some patients with varying outcomes. The tumor is low grade with no reported cases of metastases or death. The best practice for treatment is yet to be determined.