Neuromyelitis optica, a variant of multiple sclerosis, presenting with hypocalcemic tetany is an unusual presentation. We report here a case of 25 years old female who was a case of neuromyelitis optica and had hypocalcemic tetany as the initial presentation among others. The case is interesting in that the hypocalcemic tetany was not coincidental. The patient had low vitamin D status and probably, this was correlated etiologically to neuromyelitis optica. Vitamin D has immunomodulatory effect and low vitamin D status has been implicated in the etiology of autoimmune diseases such as multiple sclerosis, rheumatoid arthritis, insulin-dependent diabetes mellitus, and inflammatory bowel disease.

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