Gestational renal cell carcinoma (RCC) is an uncommon occurrence and presents a diagnostic and clinical challenge for healthcare providers. The manifestation of gestational RCC often lacks overt symptoms and can mimic physiological changes and disorders associated with pregnancy. Frequently, patients are asymptomatic, and the condition is detected during routine antenatal ultrasonography. However, the options for imaging modalities and treatment are limited due to the potential risks of harm to the developing fetus and interruption of pregnancy. Throughout the management of pregnant patients with RCC, both maternal and neonatal risks must be carefully considered, while respecting the patient's autonomy. Currently, there are no internationally or nationally recognized evidence-based guidelines for managing gestational RCC, highlighting the need for a multidisciplinary approach to treatment. Advances in surgical techniques have resulted in a shift from open surgeries to laparoscopic radical or partial nephrectomy procedures, with robotic-assisted approaches also gaining popularity. In cases of metastatic gestational RCC, termination of the pregnancy may be considered, and the appropriate treatment of RCC should be the priority. This article aims to provide a comprehensive review of the epidemiology, aetiology, clinical presentation, diagnosis, prognosis, and management of gestational RCC.