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A two-year-old female spayed Great Pyrenees dog was presented for an echocardiogram after a dilated aortic root and ascending aorta were found on a routine echocardiogram examination one year prior. A suspected aortic dissection was detected on the recheck echocardiogram, which was confirmed with cardiac magnetic resonance imaging. Subsequent genetic sequencing confirmed nucleotide variants of several connective tissue- associated proteins, of which three are known to be associated with connective tissue disorders in humans, including ADAMTS3, ADAMTSL2, and SMAD4. The dog was subclinical at the time of examination and has since been doing well with medical management on carvedilol and exercise restriction.