The different stages of cartilage development are well described but no transcription factor capable of specifically inducing differentiation to articular cartilage has been identified and little is known about the molecular mechanisms regulating cartilage development. Notch signaling is an evolutionarily conserved pathway taking part in many developmental and cell type specification processes. It has been demonstrated that markers for Notch signaling are differentially expressed during cartilage development and there is evidence for their functional role during this process. Notch signaling has further been implicated in osteoarthritis and Notch1 has been suggested as a marker for chondrogenic progenitor cells. This review summarizes the current knowledge on the role of the Notch signaling pathway in cartilage development and osteoarthritis.