A 77 year-old male presented a locally advanced small cell anal cancer and simultaneous hepatic and glandular deposits. Due to metastatic disease, chemotherapy with carboplatin and etoposide was the primary choice of treatment. Small cell cancer of the gastrointestinal tract exerts an aggressive clinical course with early metastases and a very poor prognosis. The diagnosis is based on careful histopathological examination and immunohistochemistry. Due to the aggressiveness of this tumor it is of great importance that the pathologist, the oncologist, and the surgeons are aware of this rare type of cancer.