presence of paraganglioma was ruled out. It was concluded that this was a 123 I-MIBG false-positive RCC case. Although false-positive 123 I-MIBG accumulations are very rare, they have been reported in angiomyolipoma, hepatocellular carcinoma, accessory spleen, and adrenocortical carcinoma. 2-5 To the best of our knowledge, this is the first report of a 123 I-MIBG false-positive RCC. There is a report that hydronephrosis was related to the accumulation of 123 I-MIBG in the kidney. In that case, 123 I-MIBG accumulation was observed in the renal cortex and urinoma in the kidney, in which hydronephrosis had been made by ureteric injury. 6 In this case, the affected kidney showed hydronephrosis due to tumor, which may have resulted in false positivity of 123 I-MIBG. This case was also diagnosed with Meigs' syndrome. Meigs' syndrome is a rare disorder of ovarian fibromas with ascites and pleural effusion. 7 Ascites is attributed to transudation from the surface of the tumor due to direct pressure on surrounding lymphatics and vessels. 8 The presence of ascites and ovarian tumor, coupled with increased uptake of 123 I-MIBG in kidney tumor, made us misidentify this tumor as highly malignant. The pathological diagnosis of this case was a rare variant of RCC. It is characterized by morphologically oncocytoma-like features and biologically chromophobe RCC-like features. 9,10 In this case, the differential diagnosis with SDH-deficient RCC is necessary. The mutations in SDH subunit are reported as a cause of familial paragangliomas/pheochromocytomas. SDHdeficient RCC is characterized by eosinophilic cells with clear cytoplasmic inclusions and solid architecure. 11 Morphologically, this case is different from the SDH-deficient RCC. Immunostaining of CK7, which is negative in SDH-deficient RCCs, was positive in this case. From these reasons, this case can be differentiated from an SDH-deficient RCC. The biology of this rare variant of RCC is not well understood. Further studies are warranted to clarify the characteristics of this tumor.