Conclusion (110 words):

This article presents a rare case of a patient with ADPKD suffering from both a myxoid liposarcoma and a PPGLs. Based on radiological findings and the patient’s history of severe hypertension, the lesion adjacent to the left kidney was identified as a paraganglioma. While hypertension is a common clinical feature in patients with ADPKD, this case highlights the necessity of systematically investigating secondary causes, particularly in cases of refractory or difficult-to-control hypertension. This case illustrates how an apparently straightforward clinical diagnosis can obscure a potentially serious underlying condition. Further research should explore the prevalence and optimal screening protocols for neuroendocrine tumours in ADPKD, to enhance diagnostic accuracy and patient outcomes.