ABSTRACT Background: Many studies have evaluated the spectrum of pediatric skin disorders in outpatient, inpatient, and emergency settings in different regions of Nigeria. However, literature analyzing histopathological data of dermatologic diseases in children undergoing biopsy or excision of skin lesions in Nigeria is lacking. The present study was aimed at determining the spectrum, frequency, and clinicopathologic diagnostic concordance of pediatric dermatopathological lesions at a tertiary care hospital in southeast Nigeria. Materials and Methods: This is a retrospective cross-sectional study undertaken at the department of Histopathology, Nnamdi Azikiwe University Teaching Hospital (NAUTH), Nnewi. All pediatric (up to 18 years old) skin biopsies received at the department of Histopathology, NAUTH, Nnewi over an 18-year period from January 2004 to December 2021 were included in the study. Relevant clinical and demographic data had been collected from the request forms. For ease of analysis and comparison with other skin studies, the skin lesions were classified according to the International Classification of Diseases, Tenth revision (ICD-10) (2019), for disorders of the skin and subcutaneous tissue. Results: Pediatric skin biopsies constituted 20.2% of all the skin biopsies received at our laboratory during the study period, with a male to female ratio of 1:1. The initial clinical diagnosis was concordant with histopathologic diagnosis in 88 out of 189 cases (46.6%). Forty-nine percent of biopsies/excisions were performed in the head and neck area. The most common diagnostic categories, in decreasing order of size, were neoplasms, disorders of skin appendages, papulosquamous disorders, and infections. Only about a quarter of neoplasms were malignant. Conclusion: Our study has documented the importance of histopathology as a gold standard in the diagnosis and management of skin lesions. Improved training of primary care physicians and closer cooperation between pediatricians, dermatologists, and pediatric surgeons will increase the chances of accurate diagnosis.