INTRODUCTION: Small cell carcinoma (SmCC) is a subtype of neuroendocrine tumor that has commonly been associated with lung malignancy. However, it has been seen in <1% of colorectal malignancies. Majority of neuroendocrine tumors are asymptomatic and non-functioning, but about 75% have metastatic disease by the time of diagnosis. We present a case of SmCC of the rectum in a patient who presented with intermittent hematochezia. CASE DESCRIPTION/METHODS: A 65-year-old female with a medical history of Diabetes Mellitus Type II, Hypertension, and Hyperlipidemia, was referred for endoscopic evaluation of intermittent hematochezia ongoing for several months with the following associated symptoms: change in bowel habits, constipation, perianal discomfort increased upon defecation, urinary frequency, and dysuria. She was not on anticoagulation or NSAIDs. She had no personal or family history of GI malignancy or IBD, and she denied tobacco, alcohol, or illicit drug use. Screening colonoscopy ten years ago had been negative for any abnormalities. Laboratory data was unremarkable for anemia or liver disease, but did reveal a mildly elevated CEA 4.8 ng/mL. Subsequent colonoscopy revealed large ulcerated rectal mass at 15 cm with luminal narrowing concerning for rectal malignancy. Follow up imaging with CT of the abdomen and pelvis with contrast showed a large rectal mass with multiple enlarged peri-rectal lymph nodes, 1.5 cm right adrenal nodule, 3 mm non-calcified nodule of left lower lobe and multiple sub-centimeter hypodense lesions in left liver lobe. Biopsy with histological staining suggested neoplastic cells consistent with rectal small cell carcinoma. Patient was treated with neoadjuvant chemotherapy and radiation therapy followed by abdominoperineal resection. Ultimately, she could not tolerate adjuvant therapy and enrolled in hospice. DISCUSSION: To date, fewer than 1000 cases of GI SmCC have been reported with prevalence of 0.1% to 1% of all GI tumors. Seventy- five percent of patients present with metastasis at the time of diagnosis. Six-month survival rate is 58%, and five-year survival is 6%. Clinical presentation generally mimics the usual presentation of colorectal malignancies. Workup is also similar. However, there is no standard treatment approach for SmCC as none has been developed due to low incidence. Given the paucity of literature, cases should be reported to influence future prospective studies to establish standard diagnostic and treatment regimens and to improve the overall prognosis.