A case of primary clear cell adenocarcinoma of the colon, a rare oncologic variant, was diagnosed in a 25-year-old man who presented with partial bowel obstruction. To understand better the pathology of this neoplasm, a retrospective review of Entrez PubMed entries describing primary clear cell adenocarcinoma of the colon and/or rectum was performed. Only 13 previous cases of primary clear cell adenocarcinoma of the colon and/or rectum have been reported, with an average presentation age of 57 years and generally afflicting the descending colon of men. Herein we present a case occurring in the distal ascending colon of the youngest patient in the literature to date. Our patient's diagnosis is rare in occurrence, location, and age of onset.