A case of lymphangioma of the cecum with intussusception in a 40-year-old woman is reported. She developed cramping in the right flank and came to our clinic. On her first consultation, a painful abdominal tumor was found. Abdominal ultrasonography, computed tomography and barium enema revealed an ileocecal cystic tumor accompanied by intussusception. Ileocecalectomy was performed, and during the operation incomplete fixation of colon was discovered. The tumor was dark brown, measuring 7×5×4cm and fluctuating. Macroscopically, its cut surface was cystic and it contained a yellowish, serous fluid. A histological diagnosis of cavernous lymphangioma with partial cystic degeneration was made. In reviewing the international literature, we could find only two case reports of cecal lymphangioma with intussusception. The large size of the tumor might be regarded as the cause of the complicated intussusception. However, the incomplete fixation of the colon might have been partially responsible.