Pyodermatitis-Pyostomatitis Vegetans (PD-PSV) remains a rare disorder of unknown etiology that is often associated with underlying inflammatory bowel disease (IBD). It is also worth noting that PD-PSV is particularly rare in the paediatric population with 15.4 years as the average age of onset in reported cases. Although adults and children with IBD may exhibit similar clinical features, children often manifest with additional features of failure to thrive and delayed puberty due to the background chronic inflammatory process. In this case report, we present a case of paediatric PD-PSV without underlying inflammatory bowel disease in a 3-year-old African girl. This case presents a unique opportunity of reminding clinicians that not all cases of paediatric PD-PSV may be associated with underlying IBD at presentation.