Introduction: Renal cell carcinoma (RCC) is the most common type of kidney cancer, typically presenting symptoms related to the urinary tract or metastatic spread(1). However, RCC can occasionally manifest with atypical signs and symptoms, leading to diagnostic challenges. Portal vein thrombosis (PVT) is a rare complication of RCC(2), and its presence as the initial presentation is even rarer. In this case report, we discuss a unique case in which PVT served as the presenting sign of RCC, highlighting the importance of considering RCC in the differential diagnosis of patients with PVT.