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Introduction: Intraductal papillary neoplasms of the bile duct have been recognized as a differentiated entity to other biliary tumors since 2001. They are characterized by intraductal growth, with or without mucus production, and have malignant potential, although they have a better prognosis than other types of cholangiocarcinoma. Case reports: From January 2010 to August 2015, we included three patients with a confirmed diagnosis of bile duct intraductal papillary neoplasia with malignancy that were treated at our center. Two cases were male and one female with a mean age of 67.3 years. All three patients presented malignancy at the time of diagnosis. One patient was asymptomatic and the diagnosis was reported in the hepatectomy specimen after a liver transplant due to autoimmune hepatitis. The other two patients presented with cholestasis and acute cholangitis and the diagnosis was made based on imaging tests (computed tomography [CT] and magnetic resonance imaging [MRI]) and endoscopic retrograde cholangiopancreatography (ERCP) with brushing and a biopsy. The treatment in both cases was surgical with a left hepatectomy, and resection of the left bile duct and segment I. They did not receive adjuvant treatment. None of the cases had recurrence of the disease. Discussion: Malignant intraductal papillary neoplasias of the biliary tract represent a different entity of cholangiocarcinoma with a better prognosis. Its diagnosis is based on imaging tests and histology by ERCP. The treatment is surgical, achieving a high rate of success with a low relapse rate.