She studied in Frankfurt/Main and Tübingen, earning a diploma in Biochemistry (1968) and a PhD in Genetics in Tübingen (1973).
She was a postdoctoral researcher in Basel and Freiburg, then a group leader at the European Molecular Biology Laboratory in Heidelberg (1978–1981) and at the Friedrich Miescher Laboratory of the Max Planck Society in Tübingen (1981–1984).
From 1985 to 2014, she was a scientific member of the Max Planck Society and Director at the Max Planck Institute for Developmental Biology in Tübingen.
From 2005 to 2012, she also served as Director of the Friedrich Miescher Laboratory in Tübingen.
From 2014 to 2022, she led a research group on “Colour Pattern Formation”. 

Her research focuses on the genetic and molecular analysis of embryonic development in animals, particularly the fruit fly Drosophila melanogaster and the zebrafish Danio rerio.

For her discovery of genes that control the development of animals and humans, as well as her demonstration of morphogen gradients in the fly embryo, she has received numerous awards, honorary doctorates, and prizes. These include the Gottfried Wilhelm Leibniz Prize of the German Research Foundation (1986), the Albert Lasker Medical Research Award (1991), and the Nobel Prize in Physiology or Medicine (1995). She is a member of the Royal Society (UK), the National Academy of Sciences (USA), the Order Pour le Mérite (Germany), the Leopoldina (Germany), the Berlin-Brandenburg Academy of Sciences (Germany), the Academy of Sciences (Austria), and the Académie des Sciences (France). From 2001 to 2006, she served on the German Ethics Council of the Federal Government.

In 2004, she founded the Christiane Nüsslein-Volhard Foundation to support young female scientists with children. She was President of the Society of German Natural Scientists and Physicians in 2008 and served as Secretary General of the European Molecular Biology Organization (EMBO) from 2005 to 2009. From 2005 to 2012, she was a member of the Scientific Council of the European Research Council (ERC) of the European Union. From 2013 to 2021, she was Chancellor of the Order Pour le Mérite. In 2016, she was awarded an Advanced Grant from the European Research Council (ERC).

Her hobbies include music (singing, flute, chamber music), garden design, and cooking. She is Chair of the Board of Trustees of the International Hugo Wolf Academy e.V. in Stuttgart.