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Aim: The paediatric surgical pathology specimens manifest a wide spectrum of morphological and histological features. The present work has been undertaken to know the prevalence and to describe the profile of paediatric surgical pathology specimens from western India as seen in Ahmedabad, India from 2008 to 2010. Materials and Methods: We reviewed 140 paediatric surgical specimens, 118 specimens rendered definitive diagnosis were included for the analysis. Cases were divided in two groups, one of developmental and congenital conditions and another of acquired lesions. Results: This study included 118 patients of which 79.3 % were male and 20.7 % were female. Age range of the patients was one day to twelve years. Children of one month to one year age group (infants) were the most vulnerable (31.3% cases). Group of developmental and congenital conditions consisted of 45.7% cases where as 55.3 % cases were of acquired lesions. Gastrointestinal tract was most frequently affected organ (43.2%) followed by head and neck region (14.4%) and testis (7.6%). Hirschsprung’s disease (HD) cases (6.7%) were commonest among the group of developmental and congenital conditions followed by juvenile polyps of colon (5%), Meckel's diverticulum of small intestine (5%) and neural tube defect (5%). In acquired lesions, Appendicitis was the most frequent lesion (21.2%) followed by haemorrhagic infarct of testis due to torsion (5%) and intussuception of intestine (5%). Malignant cases were (4.2%) and the most common cancer was yolk sac tumour. Conclusion: Paediatric surgical specimens, unlike adults, represent significant number of developmental and congenital conditions in addition to acquired lesions; accounting for wide spectrum of morphological and histological features. Study provides insight into the trends of paediatric surgical lesions in the western region of India.