Objectives: Rare diseases are often poorly understood, and this study sought to investigate the incidence of a rare disease entity, basaloid squamous cell carcinoma (BSCC) of the oral cavity (OC) at a tertiary care medical center and to assess its clinical outcomes. Methods: The aim of this study was to collect data in order to better understand how this rare disease progresses. This was a case series of patients with OC BSCC diagnosed between 2001 and 2018. Results: 10 patients with primary OC BSCC were identified. Average age at diagnosis was 58 years (33–71). The median follow-up period was 11 months. Primary sites included oral tongue (n = 4), floor of mouth (n = 4), hard palate (n = 1), and retromolar trigone (n = 1). A majority (60%) of patients had pathologic T3/T4 tumors. All patients underwent primary surgical treatment. There was an overall 60% mortality rate: 2 died from metastasis at 1- and 3-months postop, 2 from unknown causes, 1 from sepsis at 1 month postop, and 1 from metastatic colon cancer. Average survival for those patients who died was 20.7 months. 4 patients were disease-free at the time of publication. Conclusion: There are few studies in the literature that seek to investigate cases of OC BSCC from a single institution. This is the first detailed case series of BSCC from a single American institution. Survival outcomes in our cohort were poor but demonstrate a variable course of disease burden. This study presents unique information regarding specific pathologic characteristics and patient outcomes for this rare disease.