Intestinal-type sinonasal adenocarcinoma (ITAC) is a rare epithelial cancer of the nasal cavities and paranasal sinuses. Exposure to wood dust particles is a strong etiological factor making it a professional disease. These tumors are locally aggressive with frequent local recurrences in up to 50% of cases. Metastasis to regional lymph nodes and distant metastasis are less frequent (10%). Invasion of the duramater and local recurrence are frequent and the major cause of death. Standard therapeutic modalities include surgery followed by radiotherapy in advanced stages, sometimes with chemotherapy treatment. The molecular genetic mechanisms underlying the development and progression of this tumor is not understood. Histopathologically, ITAC resembles colorectal adenocarcinoma and have directed early genetic studies to search for similar genetic alterations. Recently, genome-wide studies have identified a recurrent pattern of chromosomal aberrations. This review aims to describe the clinico-pathological characteristics of this relatively unknown tumor and to summarize the knowledge on genetic and chromosomal analyses up to the present time.