Introduction: Lymphangiomas of the gallbladder in adults are extremely rare with only 10 cases published worldwide to date.
Case presentation: We herein report a case of a 26-year-old male who presented with abdominal right upper quadrant pain, nausea, vomiting, and diarrhea. An ultrasound, computer tomography scan, and magnetic resonance imaging showed a cystic mass interposed between the gallbladder and the liver with characteristics identical to those of lymphangiomas in more common locations. The patient underwent an open excision of the large spongy mass en bloc with the gallbladder.
Conclusion: Histological findings confirmed the diagnosis of lymphangioma. Also, we provide a review of 10 cases presented in the literature, with a discussion of the clinical features, diagnosis, and surgical approach.