BACKGROUND Adrenal incidentalomas are tumors that are usually detected in imaging studies indicated without suspicion of adrenal disease. Pheochromocytoma is a neuroendocrine tumor that can occur sporadically or associated with genetic syndromes, having been described in 0.1-5% of patients with type 1 neurofibromatosis, which is a progressive multisystemic disease of autosomal dominant inheritance that affects 1 / 2600-3000 individuals . METHODS We present the case of a 50-year-old patient who was admitted to the Emergency Department for acute appendicitis and whose CT scan showed the presence of bilateral adrenal masses is reported. After the surgical intervention, he was hospitalized for study. The patient denied headache, sweating, hypertension, palpitations, and weight loss; Physical examination revealed the presence of multiple lentigines, café-au-lait spots > 15 mm, fixed and pedunculated nodular lesions, compatible with focal neurofibromas, without signs suggestive of hypo or hypercortisolism. In the analysis, total metanephrines and vanillyl-mandelic acid were identified in urine, they were found in very high values, being subjected to laparoscopic adrenalectomy in 2 stages, after adequate preparation that included alpha and beta adrenergic blockade. CONCLUSIONS In patients with adrenal masses, it is necessary to recognize the importance of a comprehensive clinical evaluation to guide an adequate diagnostic study, as well as the performance of an optimal preoperative study that includes hormonal tests to rule out functionality.