Abstract-A 45 year old male, non vegetarian by diet, loader by occupation, presented with complaints of progressive muscle wasting and asymmetric weakness of the upper limbs of one month duration, which was followed by similar signs and symptoms in the lower limbs. There was decreased power in the affected limbs. He occasionally had muscle fasciculations. The sensory, bladder, bowel and cognitive functions were well preserved. There was no difficulty in breathing, speaking and swallowing. On the basis of history and neurological examination, a diagnosis of Motor Neuron Disease was made. All the basic investigations were found to be normal. For a definitive diagnosis, muscle biopsy was taken from various sites and to our surprise, it showed many sarcocysts filled with banana shaped bradyzoites of Sarcocystis species. Genus Sarcocystis belongs to the phylum apicomplexa and the class Coccidiea. Muscular sarcocystosis is so rare in India. Sarcocystosis presenting like a Motor Neuron Disease is a diagnostic challenge to the practitioners. The case is presented here for its rarity and to create awareness about the Sarcocystis infection and its presentation like a Motor Neuron Disease.