Acrokeratosis paraneoplastica is a rare and obligate paraneoplastic dermatosis most commonly associated with squamous cell carcinoma of the upper aerodigestive tract and lung. It has also been linked to adenocarcinomas of the lung, colon, and stomach. However, reports of its association with esophageal adenocarcinoma are exceedingly rare, with only one documented case to date. This condition primarily affects middle-aged White men, with limited cases reported in women. Moreover, its occurrence in the Chinese population is uncommon. We herein present a case of acrokeratosis paraneoplastica in a Chinese woman with primary esophageal adenocarcinoma, along with a review of the relevant literature. To our knowledge, this is the first documented instance of paraneoplastic acrokeratosis associated with esophageal adenocarcinoma in a woman.