The aim of this presentation is to review the current literature on Carcinoma ex pleomorphic adenoma (CXPA) with particular emphasis on the early changes of malignant transformation and discuss its molecular, clinical and therapeutic implications. We performed histopathology and literature review of patients with CXPA and divided them in two prognostically relevant categories widely invasive and early CXPAs. In the category of early CXPAs we included both DCIS-like and micro-invasive-like carcinoma still confined in the capsule of PA and carcinomas with extension beyond the capsule of PA of no more than 6 mm. This review suggests a wider pattern of histological appearances with gene mutation and amplification involved in the progression of CXPA and a higher prognostic threshold of about 6 mm of extracapsular invasion to be used to separate clinically favourable from unfavourable cases of CXPA.