Pseudoverrucous papules and nodules (PPN) occur in the setting of chronic irritant contact dermatitis commonly due to urine or feces. PPN most commonly affects infants but has also been reported in association with urostomies, colostomies, chronic urinary incontinence, encopresis, chronic fecal incontinence, spinal cord injury, and potentially spinal dysraphism. Herein, we present the case of a patient with spina bifida in association with Dubowitz syndrome who presented with perianal PPN. To our knowledge, there is only one other case reported in the literature of a patient with spina bifida who presented with PPN.