OBJECTIVE Acquired cystic kidney disease-associated renal cell carcinoma (ACKD-RCC) is a relatively recently described entity with scarce literature describing its imaging appearance (1, 2). The purpose of this study was to determine typical and potentially unique features of ACKD-RCC on CT scan that could aid lesion identification. METHODS A retrospective review of the CT scans of 24 patients with 29 histologically proven ACKD-RCC lesions was performed. Imaging features were recorded based on consensus readings of two radiologists. RESULTS Tumors ranged in size from 1.2 to 5 cm. Nineteen lesions were right-sided and 10 left-sided. Nineteen lesions were exophytic. One patient had bilateral lesions and three patients had multiple lesions in the same kidney. All lesions had well-defined margins with 21 round, 7 lobulated and 1 crescentic in shape. On non-enhanced exam 4 lesions were hypodense, 16 iso-dense and 9 hyperdense; 5 had gross calcifications. Twenty two patients had contrast-enhanced CT exams, with 13 lesions demonstrating homogeneous enhancement (solid pattern) and 14 having inhomogeneous enhancement (cystic or mixed solid and cystic pattern). Only 1 patient had metastatic disease. Eight patients had a history of renal transplants. CONCLUSIONS ACKD-RCCs are well-defined lesions of variable size that are almost always rounded and most often exophytic. They occasionally have calcifications and are not uncommonly hyperdense on non-enhanced exam. They are most often iso-dense on non-enhanced exam and can be solid, cystic or mixed in attenuation on enhanced exam.