Background. Unilateral basal ganglia calcinosis (BGC) is a rare radiological finding that can be diagnosed on CT and MRI but often presents challenges for clinicians and radiologists in determining its underlying cause. So far, only a few potential causes that could explain unilateral basal ganglia calcification have been described in the literature. Case Report. A 54-year-old Caucasian male was admitted to a tertiary university hospital due to the sudden onset of speech impairment and right-sided weakness. The patient had no significant medical history prior to this event. Non-enhanced computed tomography (NECT) of the brain revealed no evidence of acute ischemia, CTA shows acute MCA M2 segment occlusion. CT perfusion (CTP) maps reveal an extensive penumbra-like lesion, which is potentially reversible upon achieving successful recanalization. However, a primary neoplastic tumor with calcifications in the basal ganglia was initially interpreted as the potential cause, therefore, acute stroke treatment with intravenous thrombolysis was contraindicated. A follow-up CT examination at 24 hours revealed an ischemic lesion localized to the left insula, predominantly involving the left parietal lobe and the superior gyrus of the left temporal lobe. Subsequent gadolinium-enhanced brain MRI revealed small blood vessels draining into the subependymal periventricular veins on the left basal ganglia. Digital subtraction angiography was conducted, confirming the diagnosis of venous angioma. Conclusion. Unilateral BGC caused by venous angioma is a rare entity with unclear pathophysiological mechanisms and heterogeneous clinical presentation. It may mimic conditions such as intracerebral hemorrhage or hemorrhagic brain tumors, complicating acute stroke management, as demonstrated in this case. Surrounding tissue calcification may provide a valuable radiological clue in diagnosing venous angiomas DVAs and vascular malformations.