CLIPPERS is a rare subacute inflammatory disorder of the CNS, with a poorly understood pathogenesis and with heterogeneous clinical manifestations. Reports of extra-pontine involvement in this syndrome are rare, and as far as we know there are only two cases reported with simultaneous involvement of the CNS and pulmonary parenchyma. We report the case of a young patient with a subacute condition of cognitive dysfunction, as well as motor, sensory and balance changes. Imaging showed pontine and extrapontine brain involvement in association with multiple pulmonary nodules, characterizing a very unusual presentation of this syndrome. It is important for radiologists to be aware of that rare presentation in order to make a prompt diagnosis of this condition, enabling early treatment and avoiding permanent neurological sequelae.