BACKGROUND Extrapulmonary small cell carcinoma (SmCC) is a relatively rare clinical entity constituting only 2.5-5% of SmCCs. Recently, evidence has emerged that high-risk types of human papillomavirus (HPV) might play an etiologic role in oropharyngeal SmCC, similar to squamous cell carcinoma. CASE REPORT Here, we present a case of tonsillar SmCC that presented as combined SmCC-squamous cell carcinoma in a cervical lymph node, raising the possibility that the SmCC-component represents disease progression. CONCLUSIONS This case lends further support to the importance of HPV in the development of oropharyngeal SmCC and suggests a mechanism of disease progression.