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Cysticercosis, a major health issue in developing countries, is caused by the larval stage of Taenia solium. Disseminated cysticercosis (DCC), which is characterized by widespread cysticerci in various tissues, is rare and often asymptomatic. Here, we report the case of a 50-year-old man from rural Nepal with distal cholangiocarcinoma and DCC involving the skin, brain, orbit, tongue, soft palate, heart, and abdominal organs. Despite the presence of abdominal pain, obstructive jaundice, anemia, and significant weight loss—symptoms indicative of biliary malignancy—there were no symptoms typical of DCC. Diagnostic imaging confirmed DCC and stomach-preserving pancreaticoduodenectomy was performed. Histopathological examination of the periampullary mass revealed distal cholangiocarcinoma. Postsurgical treatment for DCC included steroids, carbamazepine, and antiparasitic therapy with albendazole. The coexistence of cysticercosis and neoplasia, though uncommon, necessitates thorough diagnostic evaluation. This case underscores the clinical complexity and highlights the need for comprehensive management of concurrent conditions.