A case is presented of a 36-year-old male with primary sclerosing cholangitis-associated inflammatory bowel disease(PSC-IBD) and two synchronous stage 1 adenocarcinomata of the colon,who was initially treated with a subtotal colectomy with ileostomy.One year later,the patient presented with extensive intra-abdominal lymphadenopathy and peritoneal carcinomatosis,as well as a markedly elevated serum level of alpha-fetoprotein(AFP).Fine needle aspiration biopsy of a porta hepatis lymph node revealed a metastatic hepatoid adenocarcinoma.Subsequent review of the previous colectomy specimen showed that one of the previously identified adenocarcinomata had features suggestive of a hepatoid colonic adenocarcinoma.The patient was subsequently treated with a cytotoxic regimen of FOLFOX(oxaliplatin,leucovorin,5-fluorouracil) and bevacizumab,with stable results being achieved after six months.This case presents the first known report of PSC-IBD associated with synchronous typical and hepatoid adenocarcinomata of the colon and highlights the importance of considering hepatoid adenocarcinoma as a differential diagnosis in patients with an increasing serum AFP level. A case is presented as a 36-year-old male with primary sclerosing cholangitis-associated inflammatory bowel disease (PSC-IBD) and two synchronous stage 1 adenocarcinomata of the colon, who was initially treated with a subtotal colectomy with ileostomy. One year later , the patient presented with extensive intra-abdominal lymphadenopathy and peritoneal carcinomatosis, as well as a marked elevated elevated serum level of alpha-fetoprotein (AFP). Fine needle aspiration biopsy of a porta hepatis lymph node revealed a metastatic hepatoid adenocarcinoma. Subsequent review of the previous colectomy specimen showed that one of the previously identified adenocarcinomata had features suggestive of a hepatoid colonic adenocarcinoma. the patient was treated with a cytotoxic regimen of FOLFOX (oxaliplatin, leucovorin, 5-fluorouracil) and bevacizumab, with stable results being achieved after six months.This case presents the first known report of PSC-IBD associated with synchronous typical and hepatoid aden ocarcinomata of the colon and highlights the importance of considering hepatoid adenocarcinoma as a differential diagnosis in patients with an increasing serum AFP level.