Introduction: Non-melanocytic skin cancers represent the most frequent type of cutaneous carcinomas. Also called basosquamous cell carcinoma and considered by some authors as a clinical form of basal cell carcinoma, metatypical carcinoma represents a controversial clinical entity.
Case presentation: This paper aims to present the case of a 42-year-old female patient who presented to the doctor’s office for the appearance of a painful cutaneous tumor located in the left submandibular region. Excisional biopsy was performed. The microscopic features were consistent with the diagnosis of metatypical carcinoma with the predominance of the squamous type.
Conclusions: Taking into consideration its aggressive behavior, careful follow-up of patients diagnosed with this rare type of cutaneous tumor is mandatory for precocious identification of possible metastases and improvement of long and short term prognosis.