Paraneoplastic acrokeratosis of Bazex (PAB) or Bazex's syndrome is a rare, paraneoplastic syndrome that mostly affects men over 40 years old, and generally associates with squamous cell carcinoma of the upper aerodigestive tract. We describe a 57-year-old woman with the characteristic features of PAB, including violaceous erythematous, scaling eruption, palmoplantar keratoderma and nail dystrophy. Further analysis revealed the underlying neoplasm to be cholangiocarcinoma. The clinical aspects of PAB and associated neoplasms are reviewed.