Background Stoma site carcinomas are rare tumors, most of which are metachronous cancers of the colon. Due to the rarity of the condition, evidence-based guidelines are yet to be formulated. The patient and the enterostomal therapist must be aware of stomal tumors to facilitate early intervention, as most tumors are diagnosed at an advanced stage. Therefore, here we describe a case of stomal carcinoma post abdominoperineal resection and its successful management. We also review similar cases reported in the literature. Case A 72-year-old male presented with a nodule over the stoma site of two months duration. He had undergone abdominoperineal resection with end descending colostomy in 1970 for rectal cancer. Examination revealed multiple nodular ulceroproliferative lesions from 9 o'clock to 3 o'clock position. Colonoscopy and staging with whole-body positron emission tomography combined with a contrast-enhanced computed tomography (PET-CT) ruled out other lesions and lymph nodal or distant metastases. Wide local excision and refashioning of stoma were performed. Histopathological examination revealed stage I metachronous descending colon cancer. His postoperative course was uneventful, and there is no evidence of tumor recurrence at three years of follow-up. Conclusion A primary adenocarcinoma originating from a colostomy site is rare but can occur many years after the end of the recommended postoperative follow-up period. It is essential to train ostomates to monitor the stoma for possible tumors to facilitate early diagnosis and to recognize the impact of such a recurrence on survival and quality of life.