We here document two cases of von Recklinghausen's disease with both pheochromocytomas and gastrointestinal stromal tumors (GIST). It is very rare that these three disorders are found to occur simultaneously, although the fact that preoperative detection of GIST is difficult except with large tumors may be important in this respect. In the present cases, GIST were not evident on preoperative computed tomography and magnetic resonance imaging, and were identified unexpectedly during surgery. Our findings indicate that investigation of the intraperitoneal space should be performed during adrenalectomy for pheochromocytomas with von Recklinghausen's disease.