Cerebellar developmental venous anomalies (CDVA) are benign conditions, although sometimes they are associated with haemorrhages and reported to be symptomatic. This is the largest follow-up study to investigate the symptomatology of CDVAs and their association with other malformations. Thirty-two patients were followed for 2 - 9 years. Twenty-eight had isolated asymptomatic CDVA without any neurological condition during follow-up, which might be linked to the CDVA. Four patients had CDVA and an associated vascular pathology: two pontine cavernomas, one asymptomatic arteriovenous malformation (this is the first published case in the literature) and one cerebellar infarct with a developmental variation of the posterior fossa venous circulation. One patient had two CDVAs, while another had a unique draining vein from the upper part of the brainstem too. In conclusion, CDVAs are benign, asymptomatic conditions, but they are sometimes associated with pathogenic malformations requiring detailed neuroradiological investigations.