Porokeratosis ptychotropica (PP) is a rare variant of porokeratosis characterized by pruritic, scaly papules and plaques localized to the perianal and gluteal cleft regions. Clinically, PP resembles other common disorders, resulting in frequent misdiagnosis. The diagnosis of PP often takes several years to make, therefore many reported cases in the literature described the late stages of PP. We report a case of PP diagnosed at an early stage. By presenting our patient, we aim to raise further awareness of PP to avoid a delay in diagnosis, thus preventing long term complications of this rare entity.