Review Article AJODRR (2020) 3:34 DIFERENTIAL DIAGNOSES OF ANGIOKERATOMAS Angiokeratomas (AK) are probably the vascular lesions that induce more confusion in the literature. The most accepted classification of AK was performed by Imperial and Helwig [1] , and they divide these lesions into five classical types: Mibelli's AK, Fordyce's AK, corporis diffusum AK, circumscribed naeviforme AK and solitary or multiple adquired angiokeratomas. Nevertheless, in the clinical practice, is not unusual to see AK type lesions associated to different types of vascular anomalies, and these lesions have difficult handling. In ISSVA classification AKs have been included as vascular anomalies provisionally unclassified. We perform the differential diagnoses of AKs and according to clinical picture, histopathological aspect, immunohistochemical markers and radiological findings proposed to divide AKs mainly in two groups. Primary AKs are the classical types. The new inmunohistochemical findings suggest that these lesions might be included as mixed capillary-lymphatic malformations. Secondary AKs are related with different vascular anomalies, secondary to other process with lymphatic obstruction, related to drugs, or associated with no vascular lesions as lymphoid lesions. Different underlying vascular anomelies might be related with AKs including deep capillary-lymphatic malformations (CLM), venous-lymphatic malformations (VLM), capillary-lymphatic-venous malformations (CLVM) (Klippel-Trenaunauy Sd), deep lymphatic malformations (LM), venous malformations (VM) as hyperkeratotic venous malformation, cavenomatous cerebral malformations, traumatic arteriovenous fistula and eccrine angiokeratomatous hamartoma. Clinical aspect, radiological studies and histopathological examination might help to do a correct diagnosis of this heterogenous entity.