Background: Sacrococcygeal Teratomas (SCTs) are germ cell tumors arising from sacrococcygeal region containing derivatives of all three germ cell lines. It is predominantly affecting neonates, infants and children along with female preponderance. Classifying SCTs according to its location and histological grading in respect to presence of immature elements are helpful in early diagnosis and therapeutic decisions, providing better outcome. Aims and objectives: Study of histological evaluation and grading of sacrococcygeal teratoma as well as correlation with clinical aspects in paediatric patients. Material and method: A retrospective study of 22 patients presenting with sacrococcygeal mass at B.J.M.C, Civil Hospital, Ahmedabad over a period of three years from 2014 to 2016. All specimens of resected saccrococcygeal mass were received and overnight fixation in 10% formalin was done. Gross examination findings were noted followed by routine paraffin embedding and tissue sectioning. Slides were stained using H&E stain, examined microscopically and grading of SCTs was done along with clinical correlation followed by comparison with other studies. Results: In present study, 22 cases which presented with sacrococcygeal mass were evaluated. There were 15 girls and 7 boys (male:female ratio of 1:2) with the age ranging from 1 day to 5 years. Majority of the cases (18(82%)) presented in infants below 2 months of age. One patient presented with recto-vaginal fistula and another with urinary retention. 19 cases were of mature teratoma (grade 0) and 3 cases were of immature teratoma (grade I) including one case of malignant transformation with yolk sac differentiation (grade II), which correlated with elevated serum AFP levels. On microscopic examination, mature teratoma showed ectodermal, endodermal and mesodermal differentiation while immature teratoma showed immature neuroepithelial elements. Conclusion: Sacrococcygeal teratoma is the commonest congenital neoplasm presenting at birth as a sacrococcygeal mass with female preponderance. Many of them are presenting with associated anomalies or constipation and urinary retention due to mass effects. When diagnosed early at birth, majority of them are of benign mature type, so that meticulous search for immature or malignant component has to be undertaken. However early diagnosis is helpful in therapeutic decisions, providing better outcome.