The porphyrias are a group of disorders of the heme biosynthesis pathway that present with acute neurovisceral symptoms, skin lesions or both. Porphyria cutanea tarda, presenting as a non-acute form, is the most common type of porphyria that encompasses a group of related disorders, all of which arise from deficient activity of the heme synthetic enzyme, uroporphyrinogen decarboxylase, in the liver. In the literature, concomitant presentation of porphyria with hepatocellular carcinoma is common; however, no case of porphyria cutanea tarda associated with cholangiocarcinoma has been seen. Here, we present a case of porphyria cutanea tarda seen in the course of cholangiocarcinoma, which can be attributed to a paraneoplastic syndrome. Our case is of interest because of its rarity. We also give a brief review of the literature regarding porphyria and cholangiocarcinoma.