INTRODUCTION: Lymphangioma is a benign congenital malformation of the lymphatic system that is relatively common in juvenile population. Rarely, it can be seen in adults and majority of the cases are reported in the head and neck region (70-75%). Typically, these malformations are asymptomatic. In this report, we present a rare case of splenic lymphangioma presenting with severe, intractable abdominal pain. CASE DESCRIPTION/METHODS: A 50-year-old female presented with three-month history of progressive abdominal pain located below the left subcostal margin. Initial vital signs and lab work were unremarkable. Physical exam was significant for tenderness in the left upper abdominal quadrant. Computed tomography (CT) scan of abdomen with contrast revealed a hypointense lesion measuring up to 1.8 cm in the posterior aspect of the spleen. Subsequently, a hypo-enhancing splenic mass in the corresponding location was demonstrated in T1-weighted MRI images (Image 1A). The spleen was normal-sized and there were no liver lesions. A total splenectomy was performed with resolution of the pain during the hospitalization. Histopathological examination of the lesion revealed multiple cystic cavities containing pale eosinophilic fluid (Image 1B) resembling lymph, consistent with splenic lymphangioma. The cystic cavities were lined with elongated cells with bland nuclei and scant cytoplasm (Image 1C). Immunohistochemically, the lesion stained positive with CD31 (Image 1D). DISCUSSION: Clinical presentation of splenic lymphangioma varies depending on the spleen size with majority of the patients being asymptomatic. The diagnosis is usually made incidentally on routine imaging studies. Based on the clinical data and imaging results, our differential diagnosis included hemangioma, complex splenic cyst, lymphangioma, lymphoma, and pseudocysts. The splenic lymphangioma was diagnosed on microscopic examination following a splenectomy which was done to avoid any further complications such as; infection, rupture, hemorrhage and pleural effusions. The treatment modality of choice was complete surgical resection in this patient given relevant history. Other therapeutic modalities such as aspiration or drainage have not shown effective results in the literature. Despite the rarity, early recognition of these lesions is important as the delay in surgical intervention can result in complications.