Among cystic tumores arising in the pancreas, microcystic adenoma is relatively uncommon;it is usually benign, and is comprised of cysts that vary in size from microscopic to 2 cm in diameter. It has recently been reported to be associated with other pancreatic tumors with malignant potential;in particular, microcystic adenoma with coexustent islet cell tumor has been reported in von Hipple-Lindau disease. We report a case of microcystic adenoma of the pancreas associated with corxistent surgically-proven islet cell tumor. On spiral CT, the islet cell tumor was seen as a highly enhanced inhomogeneous solid mass in the pancreatic head, and microcystic adenoma as numerous small cysts throughout the pancreas.