The chapter titled “Rodent Models of Recessive Cerebellar Ataxias and Spontaneous Cerebellar Mutants” by Aditya Singh, Ph.D. and Paul Mathews, Ph.D. explores the use of rodent models to study autosomal recessive cerebellar ataxias. The chapter begins by emphasizing the importance of understanding the genetic and molecular basis of these ataxias for developing treatments. It highlights the significance of mouse models in uncovering disease mechanisms and gene functions, particularly focusing on cerebellar mutant mice. The chapter is divided into sections on targeted disease models and classical spontaneous mutant models. Targeted disease models mimic the genetic basis of human diseases through genetic modification technologies, helping to elucidate the roles of associated genes and proteins in disease development and progression. Classical spontaneous mutant models arise by chance or induced mutagenesis without targeting specific genes. These models provide insights into the genetic variability and complexity of cerebellar ataxias. The authors discuss the strengths and weaknesses of each model, their contributions to understanding disease mechanisms, and their use as preclinical tools for evaluating therapies. The chapter concludes with a discussion on the challenges and future directions in the field, emphasizing the need for refined models that better replicate human disease complexity.