Objectives: Limb-girdle muscular dystrophy autosomal recessive type 4 (LGMDR4) earlier designated as LGMD2E is a rare genetic disease due to the mutation in the sarcoglycan gene (SG). To identify the mutation type in LGDMR4 in Sathwara community, India. Methods: Molecular diagnosis involving NGS was carried out on blood samples using Illumina MiSeq. STRAND NGS software was used for the alignment and variant calling and StrandOmics variant annotation engine was used for the variant reporting. SGCB protein structure model was based on comparison of primary sequences with nearest available known structure. The converted amino acid sequence of NM_000232.5 in Fasta format was submitted to YASARA structure software. Results: We report here the clinical and genetic data from children of three unrelated families belonging to the Sathwara community of Gujarat, India diagnosed with a relatively rare subtype of LGMDR4 (β-sarcoglycan, SGCB) with the same homozygous missense mutation (Chr 4:52894204C>T; c.683G>A; p.Gly228Glu) in the SGCB gene (exon 5). Conclusion: LGMDR4 maybe a common muscular dystrophy among the Sathwara community, and further studies on this population is suggested for determining the basis of natural history of this disease. The identified novel missense mutation present may result in a conformational change in the extracellular domain of the SGCB protein leading to a modification of sarcoglycan complex. These results indicate high prevalence of this mutation in this community with founder effect.