Microphthalmia-associated transcription factor family translocation renal cell carcinoma (MiT-tRCC) stands out as a rare subtype of kidney cancer with distinct biological features compared to other kidney cancer subtypes. It encompasses TFE3-rearranged RCC (also known as Xp11 translocation RCC) and TFE-rearranged translocations RCC, although multiple new fusion partners were identified. Traditionally thought to primarily affect children and young adults, more cases of MiT-tRCC are being identified in adults. It was first officially recognized in the 2004 World Health Organization (WHO) renal tumor classification and recently TFE3 (Xp11) rearrangement and TFEB alterations were included in the WHO 2022 "molecularly defined renal carcinomas" as a distinct group. This subtype is distinguished by gene fusions involving the MiT family of transcription factors. Recent strides in diagnostic and molecular sequencing assays have significantly enhanced our comprehension of these tumors, uncovering novel and distinct molecular features. The discovery of novel immune-checkpoint inhibitors and anti-angiogenic targeted therapies has notably broadened the therapeutic options for clear cell RCC. These advancements have prompted the consideration and study of these innovative therapies in translocation RCC. In this review, we offer an overview of translocation RCC and delve into the current strides in the management of this distinctive disease, highlighting the integration of recent breakthroughs in therapeutic approaches.