BACKGROUND: Inflammatory bowel disease (IBD) can occur at any age. In pediatric patients, the disease may present with a broader range of symptoms and more severe course than in adults, due to ongoing growth and development. Therefore, pediatric IBD often exhibits an atypical clinical course and laboratory findings. It is essential to recognize differences in disease presentation, differential diagnoses, and evaluation strategies specific to children. The revised Porto criteria, proposed by the European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) in 2014, are widely used globally, including in Japan, for the diagnosis of pediatric IBD. PURPOSE: Despite the widespread use of these criteria, no formal diagnostic guidelines for pediatric IBD have been developed in Japan. We aimed to support future guideline development by summarizing important diagnostic considerations and clinical practices for pediatric IBD in Japan. METHODS: This review was developed based on relevant international diagnostic guidelines and the expert opinions of Japanese pediatric gastroenterologists. It outlines key clinical and laboratory evaluations, as well as current treatment and follow-up approaches. RESULTS: We summarized recommended diagnostic tests and clinical points that require special attention in children with suspected IBD. The article reflects both global standards and domestic clinical experience. CONCLUSION: Although this article does not provide formal diagnostic criteria or assess evidence levels, it offers accurate and practical information to guide physicians and patients in the diagnosis and management of pediatric IBD in Japan.