Abstract
Splenic lymphangioma (SL) is a rare benign lymphatic malformation resulting from abnormal development of lymphatic vessels. It primarily affects children and may coexist with lymphatic malformations at other anatomical sites. Most patients are asymptomatic, and the diagnosis is often incidental. When symptoms occur, they are usually related to the size of the lesion and the compression of adjacent organs. We present the case of a 14‐year‐old male with a history of congenital gluteal and perineal lymphatic malformations, initially misdiagnosed prenatally as myelomeningocele. During follow‐up, the patient developed persistent colicky abdominal pain, progressive abdominal distension, and cutaneous lesions resembling frogspawn, exuding purulent discharge. Physical examination revealed a large, firm, and painful abdominal mass extending from the epigastrium to the hypogastrium. A contrast‐enhanced abdominal computed tomography (CT) scan revealed massive splenomegaly with multiple nonenhancing cystic lesions and displacement of adjacent structures. An open splenectomy was performed due to symptomatic mass effect and diagnostic uncertainty. Intraoperatively, a markedly enlarged, multilobulated spleen was identified and removed without complications. Histopathological examination confirmed the diagnosis of SL, showing multiple thin‐walled cystic spaces filled with eosinophilic proteinaceous material and lined by flat endothelial cells. The patient had an uneventful postoperative course, completed postsplenectomy prophylaxis, and was discharged in stable condition with good oral tolerance, normal bowel and urinary function, and no signs of infection or surgical complications. This case highlights the importance of considering lymphatic malformations in the differential diagnosis of pediatric patients presenting with atypical splenomegaly and cutaneous lymphatic lesions. Early diagnosis and appropriate surgical management can offer both symptomatic relief and definitive diagnostic confirmation in selected cases.