Nüsslein-Volhard was educated at the university of Tübingen. After a spell at the European Molecular Biology Laboratory at Heidelberg, she moved in 1981 to the Max Planck Institute for Development, Tübingen, where she has served since 1990 as the director of the department of genetics.

From 1978 to 1981, Nüsslein-Volhard collaborated with Eric Wieschaus on identifying the genetic factors responsible for the development of the fruit fly Drosophila melanogaster. Large numbers of mutants were bred by feeding adult flies mutagenic chemicals. After examining many thousands of flies they had managed, by 1980 to identify the main development sequence in Drosophila.

Nüsslein-Volhard has also succeeded in illuminating the general process of development. It has long been thought that differentiation in early embryos – anterior from posterior, for example, or dorsal from ventral – was caused by varying concentration of substances along the axes of the egg. The theory of morphological gradients, as it is known, has recently been supported by experimental work carried out by Nüsslein-Volhard and her Tübingen colleagues.

Nüsslein-Volhard shared the 1995 Nobel prize for physiology or medicine with Edward Lewis and Eric Wieschaus for their work on the development of Drosophila.