Acute chylous ascites is a rarely seen clinical picture, therefore, examination findings are often confused with acute appendicitis. To the best of our knowledge, there is no publication to date showing the occurrence of them together. This study presents the treatment plan for a 25-year-old male patient with both acute chylous ascites and appendicitis. Surgical findings were retrocaecal appendicitis, evident lymphangiectasia in the proximal segment of jejunum, and approximately 3 lt of chylous fluid. An appendectomy was performed and drainage was applied. Low-fat total parenteral nutrition (TPN) and octreotide treatment were administered for 7 days postoperatively. We also present a general review of some studies on chylous ascites, which have been published in the English language medical literature since 1910.