Abstract Background and Aims Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent inherited kidney disease worldwide. It is characterized by inexorable cyst formation in the kidney and extrarenal manifestations, such as liver cysts, colonic diverticula, valve prolapse, and cerebrovascular complications. The latter is associated with high morbidity and mortality and results from a higher prevalence of cerebral aneurysms (12–25%) compared to the general population. we aimed to evaluate the clinical and imaging characteristics and prognosis of patients with ADPKD with cerebrovascular complications. Method We reviewed the electronic medical records of patients seen at the nephrology department of the Inkosi Albert Luthuli Central Hospital from 2006 to 2023. We are reporting on 13 patients with ADPKD complicated by intracerebral haemorrhage (ICH), subarachnoid haemorrhage (SAH), non-traumatic subdural hematomas (NTSDH), and unruptured intracranial aneurysms (UIAs). We extracted demographic, clinical, and paraclinical data. We performed bivariate analysis to find associated factors. Results Of the 13 patients, 8 were women and 5 men with 6 of African ethnicity, 4 Asians, and 2 Caucasians. The median age at presentation was 52 (31, 72). Vascular risk factors included hypertension (mean SBP150.3 ± 17.25, mean DBP 84.5 ± 12.2), dyslipidaemia, and smoking. Most patients presented with various levels of altered consciousness. Imaging showed 4 NTSDH, 4 ICH, 3 SAH, and 2 unruptured aneurysms. The affected vessels were the left Middle Commutating Artery, the posterior perforating artery, and the anterior choroidal artery. Follow-up duration ranged from 1 to 15 years. The NTSDH reoccurred in 2 patients. Patients with unruptured aneurysms were managed conservatively while the others benefited from a surgery after which 2 died of surgical complications. These 2 patients who died were end-stage kidney disease(ESKD) and presented with a lower Glasgow Coma Scale. In Univariate analysis cerebrovascular haemorrhage was associated significantly with tobacco (P = 0.011), younger age at diagnosis of ADPKD (P = 0.010), and higher systolic BP (P = 0.021) but not ethnicity (P = 0.106). Conclusion Patients of African ethnicity were more likely to present with NTSDH and it reoccurred. Overall surgical management led to full recovery except for patients with ESKD and those with lower GCS.