INTRODUCTION AND IMPORTANCE: Takayasu arteritis (TA) is a granulomatous, large vessel inflammation characterized by occlusion, stenosis or aneurysm of the aorta and its branches. Aortic dissection (AD) is one of the rare manifestations of TA, which can present as uncontrolled hypertension. This case illustrates the severe rare cardiovascular complication that arises in a 21-year male patient with TA, including hypertensive urgency, AD and significant renal artery involvement. CASE PRESENTATION: A 21-year-old male presented with complaints of fever, malaise and dizziness. On clinical examinations, a discrepancy in blood pressure was noted in bilateral arms. All the laboratory parameters were within normal limits. Echocardiography confirmed moderate concentric hypertrophy with Grade II diastolic dysfunction and a preserved ejection fraction. To rule out secondary causes of hypertension, an arterial Doppler was done, which showed features of renal artery stenosis and stenosis in the descending aorta. CTA revealed an intimal tear/dissection in the thoracoabdominal aorta consistent with Stanford Type B AD. CLINICAL DISCUSSION: Based on clinical presentation and imaging findings, large vessel vasculitis such as TA or giant cell arteritis (GCA) was suspected. Later, TA was diagnosed using the 2022 American College of Rheumatology/EULAR classification criteria for TA. The patient had rapid symptomatic improvement using antihypertensive drugs, corticosteroids and immunosuppressants. CONCLUSION: This explains rare manifestation of TA in a young male patient with normal inflammatory markers and underlying AD with hypertensive urgency. In those suspected with TA, screening for life threatening conditions such as AD should be done.