Primary bladder perivascular epithelioid cell tumour in an early adolescent

Primary bladder perivascular epithelioid cell tumours (PEComas) are rare tumours that typically present in the third or fourth decade of life. Radiological and histopathological findings play a crucial role in establishing the diagnosis, and treatment is primarily based on surgical excision. We present the case of a primary bladder PEComa in an early adolescent male presenting with macroscopic haematuria and a hypervascular bladder mass. These peculiar symptoms should prompt consideration of the rare diagnosis of PEComa, even in paediatric patients.