Pyostomatitis-pyodermatitis vegetans is an uncommon mucocutaneous dermatosis of unknown etiology. It presents as erythematous pustules that coalesce to form exudative vegetating plaques on the oral mucosa and/or skin. Diagnosis is largely based on clinical assessment, although histopathology should be used as supporting evidence. Pyostomatitis-pyodermatitis vegetans is associated with inflammatory bowel disease, liver dysfunction, and others. We report a case of a 48-year-old man who presented with erythematous eroded plaques involving his nares and upper lip. Investigations revealed a dense mixed inflammatory infiltrate and pronounced peripheral eosinophilia. Uniquely, the patient did not have inflammatory bowel disease or any other systemic condition. Systemic corticosteroids were helpful in clearing mucocutaneous lesions; however, this effect was only sustained at high doses. There are few reports of pyostomatitis-pyodermatitis vegetans in the medical literature. This case highlights key aspects of this extremely rare dermatosis, especially in otherwise healthy patients without inflammatory bowel disease.