Monkeypox deaths have been rarely reported across the globe. A total of 19 fatalities have been reported globally from endemic and non-endemic countries in 2022. The current outbreak of Monkeypox had been largely limited to the community of men who have sex with men primarily with multiple bisexual or homosexual partners. The vast majority of the cases infected with Monkeypox had recovered and the complications like sepsis and encephalitis had been reported in the immunocompromised individuals. The mortality in Brazil and Mexico were primarily in the immunocompromised individuals, while in Spain the fatal cases were immunocompetent with no underlying conditions. To date, India had recorded 12 cases of Monkeypox infection from Kerala (n=5) and New Delhi (n=7). Here, we report the first fatal case of Monkeypox virus (MPXV) infection imported from UAE to Kerala, India in July 2022. A 22-year-old apparently immunocompetent male with no significant past medical history was admitted in an unconscious state to a private hospital in Kerala following a single episode of acute onset generalized tonic-clonic seizures. The clinical features, MRI findings, CSF picture, and EEG findings of this case suggest encephalitis. Only the case's oropharyngeal/nasopharyngeal swabs (OPS/NPS) were found to be positive for MPXV. The next generation sequencing on OPS/NPS specimen could retrieve 92.76% of the MPXV genome and belonged to A.2 lineage of clade IIb as observed in the other confirmed Monkeypox cases from India.