Madras motor neuron disease (MMND) is a rare neurodegenerative condition first reported in Southern India. It causes motor neuron damage leading to muscle wasting in limbs, tongue shrinkage, pyramidal signs, sensorineural loss and weakness in cranial nerves like the facial and lower nerves. The review looks at 25 patients from 15 families, symptoms often start at the age of 13 on average, with disease lasting to 3-348 months. This data was analyzed by Chi-square test or Fisher’s exact probability test for categorical data. They used Student t-test to compare continuous data and Kaplan-Meier for survival analysis. The results conclude a comparative study of the clinical features between Familial MMND (FMMND) and Sporadic MMND (SMMND) and most cases follow autosomal recessive inheritance with rare dominant pattern. The discussion highlights the need for the exploration of the genetical extremes, etiological causes and therapeutic aspects. Also covering the difference between BVVL and FMMND.