Evaluating patients with intracerebral hemorrhage is common practice in the field of stroke neurology but can be complicated and may require extensive evaluation in younger patients with no history of hypertension. In this case, a healthy 32-year-old woman presented with an acute spontaneous lobar intracerebral hemorrhage. Neurologic workup required extensive imaging evaluation, genetic testing, and a thorough evaluation of patient and family medical history to identify the final diagnosis. This case highlights the diagnostic approach and importance of thorough clinical evaluation of young patients with intracerebral hemorrhages. Readers will walk through the stepwise diagnostic approach to arrive at the leading diagnosis with a review of the possible differential diagnoses and a discussion of this rare condition.