We describe the association of cerebellar ataxia and hypogonadism in four female patients. Clinical, neurophysiological, neuroradiological and endocrinological features are reported. Post-mortem findings in one case are also described. We show in our patients that, beside the cerebellum, both the supratentorial white matter and the peripheral nerve are involved. We suggest that this association can be considered as a "multiple system atrophy". Furthermore, since hypogonadism is hypergonadotropic in three patients and hypogonadotropic in one, the endocrine pattern appears to be heterogeneous.