Survivin, a member of the inhibitor of apoptosis protein family, is one of the most cancer-specific proteins identified to date. Survivin expression is low or undetectable in most adult tissues, but, alternatively, is overexpressed in a large number of tumors. This multifunctional protein is recognized as a key regulator in apoptosis, proliferation and angiogenesis in the tumor environment. Several studies have shown a correlation between survivin upregulation and poor cancer prognosis, and, as expected, its downregulation or inactivation leads to inhibition of tumor growth. Therefore, survivin has attracted increasing attention both as a potential cancer biomarker and as a new target for anticancer therapies. This review summarizes and discusses survivin expression and its potential as a prognostic and diagnostic biomarker in different types of tumors, as well as provides an overview of the current therapeutic challenges of targeting survivin as a treatment strategy.