Introduction: Kleeblattschädel (cloverleaf) skull is a very uncommon form of pansynostosis. Some cases are associated with syndromes, but often, this skull deformity is seen in isolation. To date, a comprehensive review of this topic is not available in the medical literature. Conclusions: Herein, we review the history, associations, diagnosis, treatment, and complications of Kleeblattschädel skull. © 2012 Springer-Verlag Berlin Heidelberg.