Primary angioplasmacellular hyperplasia is a scarce clinical dermatologic condition presenting different skin lesions diagnosed through biopsies indicating vascular proliferation with perivascular plasma cell infiltrations. In the current case presentation, a 40-yearold healthy male was referred to our outpatient dermatologic clinic with concurrent limbs and trunk erythematous vascular lesions for five years. The chief complaint of the patient was intermittent pruritus and relative irritation for years. Multiple biopsies were derived from the lesions, and vascular proliferation of capillaries with intensive perivascular infiltration of plasma cells was detected in the pathological specimen that finally led us to the final diagnosis of primary angioplasmocellular hyperplasia. Treatment with isotretinoin was initiated, and the patient responded to the treatment protocol dramatically. Given the rarity of reported cases, this report is considered the first case of primary angioplasmacellular hyperplasia with numerous lesions scattered and the first experience of successful treatment with isotretinoin.