en Oncology Oncology case report case report <span style="font-size:14px;"><span style="font-family:Times New Roman;"><span style="color:#0033ff;"><strong><em>Background</em></strong><em>:</em> </span>Neurofibromatosis type 1 (NF-1) is a genetic disorder. A heterogeneous group of benign and malignant neoplasms are associated with NF-1. Adrenocortical carcinoma (ACC) is an extremely rare invasive malignancy. The association of ACC with NF1 is not well understood.<br> <span style="color:#0033ff;"><strong><em>Case Presentation</em></strong><em>:</em></span> We report a case of ACC in the context of NF1 in a 39- year-old woman who referred with the chief complaint of a mass in left abdomen. A left adrenal lesion was diagnosed by CT scan. Biochemical tests showed no abnormality. Adrenalectomy was done and histological and immunohistochemical findings confirmed ACC. Due to the absence of metastasis, clinical stage II was considered for the tumor. On follow-up after six months, she was still alive and well and with no evidence of metastasis. The age of patient and lack of secretion of adrenal cortical hormones in this case were unlike most ACCs.<br> <span style="color:#0033ff;"><strong><em>Conclusion</em></strong><em>:</em></span> Also, modified Weiss score for malignancy of adrenocortical neoplasms, clinical staging system and different modality of treatment is discussed.</span></span> Adrenocortical Carcinoma, Neurofibromatosis 1, Adrenal Glands, Adrenal Cortex Neoplasms 613 617 http://caspjim.com/browse.php?a_code=A-10-2010-2&slc_lang=en&sid=1 Mohammad Najafi-Semnani abbaszadehbidokhty@gmail.com 100319475328460032176 100319475328460032176 Yes Department of Urology, School of Medicine, Birjand University of Medical Sciences, Birjand, Iran Mahdieh Rajabi-Moghaddam rajabimmh@gmail.com 100319475328460032177 100319475328460032177 No Department of Pathology, School of Medicine, Birjand University of Medical Sciences, Birjand, Iran Hamid Abbaszadeh hamidabbaszade@yahoo.com 100319475328460032178 100319475328460032178 No Department of Oral and Maxillofacial Pathology, School of Dentistry, Birjand University of Medical Sciences, Birjand, Iran