A middle-aged man presented with 1-month history of headache, dizziness, staggering of gait and progressive deafness. Examination revealed decreased visual acuity, papilloedema, gaze evoked nystagmus, bilateral sensorineural deafness and gait ataxia. MRI showed TI iso and T2 hyperintensity of cerebellum, prominence of folia with gyral enhancement in a tigroid pattern making impression of the rare entity, Lhermitte Duclos disease (LDD) or dysplastic gangliocytoma of cerebellum. Conservative management for the raised intra cranial pressure did not have any impact on patient's general condition for which an MR venogram was planned and it surprisingly showed non-visualisation of left transverse sinus and internal jugular vein, suggestive of thrombosis. Patient was treated with anticoagulants but he succumbed to his illness soon after. We present this case to convey that a high index of suspicion should always be there in mind for this commoner condition (venous sinus thrombosis) before diagnosing the rarer ones (here LDD disease) to prevent the disastrous consequences of the former.