Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 9 6 2025 07 01 266 271 Abdus Salam Raju Department of General Surgery, Upper GI Surgery Liverpool Hospital, Sydney, NSW Australia. AND Western Sydney University, Sydney, NSW Australia. Dinuke de Silva Department of Anatomical Pathology, NSW Health Pathology, Sydney, NSW Australia. Yasser Farooque Department of General Surgery, Upper GI Surgery Liverpool Hospital, Sydney, NSW Australia. Tristan Rutland Western Sydney University, Sydney, NSW Australia. AND Department of Anatomical Pathology, NSW Health Pathology, Sydney, NSW Australia. 2025 03 17 2025 04 06 Papillary Intralymphatic Angioendothelioma (PILA), or Dąbska Tumour, is an exceedingly rare lymphovascular neoplasm of intermediate malignancy, predominantly affecting cutaneous and soft tissue sites. This case report highlights an unusual presentation of PILA in the mesentery of a 38-year-old male, a site not previously documented. He initially underwent a splenectomy for symptomatic splenic hemangioma. His symptoms recurred two years later, presenting with haemoperitoneum and extensive abdominal involvement, including the colon, small bowel mesentery, and diaphragms. An en-bloc extended right hemicolectomy and partial hepatectomy achieved complete resection. Histopathology revealed hobnail endothelial cells and characteristic papillary structures, confirming PILA. Despite its rare visceral occurrence and diagnostic challenges, the patient showed no recurrence at six months post-surgery. This case underscores the importance of histopathological analysis and awareness of atypical presentations. Wider surgical excision remains the cornerstone of treatment, with vigilant follow-up essential for detecting recurrence and guiding future management protocols for this rare entity. https://crcp.tums.ac.ir/index.php/crcp/article/view/1032 https://crcp.tums.ac.ir/index.php/crcp/article/download/1032/698