Abstract Symmetrical acrokeratoderma is a recently described dermatosis in young adult males of Chinese descent. In this report, we describe a series of five cases of symmetrical acrokeratoderma from India. All 5 patients had asymptomatic, brownish-black plaques distributed symmetrically over dorsum of hand and feet with variable involvement of wrist, flexural surface of forearm, elbow, ankles, shin and knee joint. Palms and soles were characteristically spared. Typically whitish maceration of the lesions was seen after immersing in water. The disease showed exacerbation in hot and humid climate with spontaneous resolution in winter. Histopathological examination showed basket weave hyperkeratosis, irregular acanthosis and mild peri-vascular lymphomononuclear infiltrate. Loosening of the stratum corneum was seen in post-immersion biopsy specimens. Acitretin appeared to provide symptomatic improvement in the short term without any long-term effect on the natural disease course. The genetic and environmental factors involved in disease causation needs to be elucidated in future. Trial registration The study was not registered in a trial registry since it was a retrospective analysis of the clinical records and not an interventional/observational study.