Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) is considered as a rare, benign vascular lesion that mainly affects the skin and subcutaneous tissues of the head and neck region, but is rare in the oral cavity. Its etiology and pathogenesis remains unclear and its diagnosis is based on the histopathological findings. Here we report a case of ALHE showing a swelling in the oral cavity mucosa. Case description: A 24-year-old female presented to our outpatient department with the complaints of a painless swelling in the oral cavity on the right cheek since 1 month. The patient underwent excision of the swelling intraorally, and diagnosis of ALHE was made based on the histopathological findings of a chronic inflammatory infiltrate admixed with proliferating blood vessels. Conclusion: ALHE is rare in the oral cavity mucosa and can present a diagnostic dilemma. Clinical significance: ALHE should be considered as a differential diagnosis of a submucosal swelling in the oral cavity and complete excision of the swelling helps in both diagnostic and definitive treatments.