We describe a rare case of epidermoid cyst arising in an intrapancreatic accessory spleen that presented as a cystic mass in the tail of the pancreas, and for which laparoscopic distal pancreatectomy was performed successfully. A 36-year-old woman with a cystic mass in the tail of the pancreas, which had been discovered incidentally at a medical checkup, was referred to our department for further examination. Endoscopic retrograde cholangiopancreatography, endoscopic ultrasonography and positron emission tomography demonstrated a multilocular cyst in the tail of the pancreas without any evidence of malignancy, although differential diagnosis was extremely difficult because of the neoplasm-like appearance of the lesion. Therefore, we performed laparoscopic distal pancreatectomy under a preoperative diagnosis of mucinous cystic neoplasm. Postoperative pathologic examination demonstrated an epidermoid cyst arising from a heterotopic spleen within the pancreas. This is the first report of successful laparoscopic distal pancreatectomy for an epidermoid cyst arising in an intrapancreatic accessory spleen. One virtually has no chance to diagnose an epidermoid cyst in an accessory spleen on the basis of preoperative diagnostic workup, and consequently the type of surgical resection (open vs. laparoscopic) would be conditioned by factors other than the clinical entity suspected at the preoperative period.