Dioctophyma renale is classified as a cosmopolitan zoonotic helminth, belonging to Nematoda class and Dioctophymoidea superfamily. Dogs are considered definitive hosts, suffering with large kidney damage, and the disease diagnosis can be done through ultrasound, x-ray and light microscopy. The aim in this paper was to report the sonographic and microscopic aspects of a canine affected by Dioctophyma renale. Dark colored urine evolving to hematuria and abdominal pain was the main symptoms observed in the animal. Performing urinalysis it was verified D. renale eggs. Ultrasonography could observe total destruction of the right renal parenchyma, hyperechoic structures of cylindrical and rounded shape bounded by the renal capsule, suggesting the presence of the parasite, while the left kidney had normal size. The animal was submitted to nephrectomy, confirming the worm presence, measuring 31.8 cm long. It can be stated that ultrasound and light microscopy are highly effective to establish a definitive diagnosis of Dioctophyma renale