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Fibromuscular dysplasia (FMD) is a vascular disease characterized by abnormal arterial wall architecture, usually visualized angiographically as the classic “string of beads” sign. We present the diagnostic dilemma of a 51-year-old woman admitted for a spontaneous renal infarct, with initial examination and investigations equivocal. She was consequently assessed for thromboembolic and vasculitic disease, before proceeding to digital subtraction angiography (DSA). This invasive method elucidated an area of focal FMD, previously not seen on computed tomography angiography (CTA). This is unusual due to CTA's high sensitivity and specificity but could be attributed to the less common subtype of FMD which requires combined imaging modalities to arrive at a diagnosis. Hence, there may be a role for the dual use of CTA and DSA in young patients presenting with a spontaneous renal infarct in the absence of other diagnoses.