Although sporadic Creutzfeldt Jakob disease is always fatal and no accepted treatment is currently available. Moreover, prevalence of COVID-19 in the same patient makes management protocol difficult and prognosis worse.

Portal Hypertension is not a classical presentation of Caroli's Syndrome. However, some young children can present with overt signs and symptoms indicative of advanced disease state despite the improvement in imaging technology which has decreased its diagnostic age. High index of clinical suspicion can help in timely diagnosis and management